> top > docs > PMC:1448208 > spans > 32303-39439 > annotations

PMC:1448208 / 32303-39439 JSONTXT

Annnotations TAB JSON ListView MergeView

craft-sa-dev

Id Subject Object Predicate Lexical cue
T21121 6851-6855 NN denotes MSUD
T21120 6838-6850 JJ denotes intermediate
T21119 6856-6861 NN denotes model
T21118 6834-6837 DT denotes the
T21117 6832-6834 , denotes ,
T21112 6823-6825 CD denotes 36
T21111 6822-6823 , denotes ,
T21110 6820-6822 CD denotes 35
T21109 6829-6831 CD denotes 46
T21108 6819-6820 -LRB- denotes [
T21107 6809-6818 NNS denotes complexes
T21106 6797-6808 NN denotes multienzyme
T21105 6794-6796 IN denotes of
T21104 6785-6793 NN denotes analysis
T21103 6774-6784 JJ denotes structural
T21102 6771-6773 IN denotes in
T21101 6762-6770 NN denotes interest
T21100 6755-6761 JJ denotes recent
T21099 6752-6754 IN denotes of
T21098 6866-6873 VB denotes provide
T21097 6744-6751 IN denotes Because
T21096 6743-6928 sentence denotes Because of recent interest in structural analysis of multienzyme complexes [35,36,45,46], the intermediate MSUD model may provide a valuable resource for studies of structural biology.
T21095 6742-6743 . denotes .
T21094 6730-6735 JJ denotes whole
T21093 6736-6742 NN denotes animal
T21092 6726-6729 DT denotes the
T21091 6723-6725 IN denotes of
T21090 6717-6722 NN denotes level
T21089 6713-6716 DT denotes the
T21088 6710-6712 IN denotes at
T21087 6698-6702 NN denotes acid
T21086 6692-6697 NN denotes amino
T21085 6703-6709 NNS denotes levels
T21084 6686-6691 NN denotes blood
T21083 6682-6685 CC denotes and
T21082 6673-6681 NN denotes activity
T21081 6667-6672 NN denotes BCKDH
T21080 6664-6666 IN denotes to
T21079 6656-6663 NN denotes respect
T21078 6651-6655 IN denotes with
T21077 6643-6650 NN denotes thiamin
T21076 6640-6642 IN denotes of
T21075 6633-6639 NN denotes effect
T21074 6629-6632 DT denotes the
T21073 6624-6628 VB denotes test
T21072 6621-6623 TO denotes to
T21071 6615-6620 NN denotes model
T21070 6613-6614 DT denotes a
T21069 6593-6597 NN denotes MSUD
T21068 6580-6592 JJ denotes intermediate
T21067 6598-6603 NN denotes mouse
T21066 6576-6579 PRP$ denotes our
T21065 6574-6576 , denotes ,
T21064 6560-6565 JJR denotes newer
T21063 6566-6574 NNS denotes findings
T21062 6554-6559 DT denotes these
T21061 6551-6553 IN denotes of
T21060 6545-6550 NN denotes light
T21059 6604-6612 VBZ denotes provides
T21058 6542-6544 IN denotes In
T21057 6541-6743 sentence denotes In light of these newer findings, our intermediate MSUD mouse provides a model to test the effect of thiamin with respect to BCKDH activity and blood amino acid levels at the level of the whole animal.
T21056 6540-6541 . denotes .
T21055 6539-6540 -RRB- denotes ]
T21054 6536-6537 , denotes ,
T21053 6534-6536 CD denotes 45
T21052 6537-6539 CD denotes 46
T21051 6533-6534 -LRB- denotes [
T21050 6515-6525 VBG denotes expressing
T21049 6512-6514 NN denotes E2
T21048 6526-6532 NN denotes allele
T21047 6502-6507 RBS denotes least
T21046 6508-6511 CD denotes one
T21045 6499-6501 RB denotes at
T21044 6496-6498 IN denotes of
T21043 6487-6495 NN denotes presence
T21042 6483-6486 DT denotes the
T21041 6478-6482 IN denotes upon
T21040 6468-6477 JJ denotes dependent
T21039 6443-6444 HYPH denotes -
T21038 6444-6454 JJ denotes responsive
T21037 6436-6443 NN denotes thiamin
T21036 6455-6464 NN denotes phenotype
T21035 6432-6435 DT denotes the
T21034 6465-6467 VBZ denotes is
T21033 6427-6431 IN denotes that
T21032 6412-6416 VBP denotes have
T21031 6406-6411 NNS denotes cells
T21030 6401-6405 NN denotes MSUD
T21029 6396-6400 IN denotes with
T21028 6417-6426 VBN denotes suggested
T21027 6380-6387 NN denotes culture
T21026 6375-6379 NN denotes cell
T21025 6388-6395 NNS denotes studies
T21024 6368-6374 JJ denotes Recent
T21023 6367-6541 sentence denotes Recent cell culture studies with MSUD cells have suggested that the thiamin-responsive phenotype is dependent upon the presence of at least one E2 expressing allele [45,46].
T21022 6366-6367 . denotes .
T21021 6365-6366 -RRB- denotes ]
T21020 6364-6365 CD denotes 1
T21019 6363-6364 -LRB- denotes [
T21018 6355-6362 NN denotes success
T21017 6347-6354 JJ denotes limited
T21016 6334-6337 VBZ denotes has
T21015 6328-6333 NNS denotes doses
T21014 6320-6327 NN denotes thiamin
T21013 6317-6319 IN denotes of
T21012 6306-6310 JJ denotes wide
T21011 6311-6316 NN denotes range
T21010 6304-6305 DT denotes a
T21009 6299-6303 IN denotes with
T21008 6338-6346 VBN denotes produced
T21007 6289-6298 NN denotes treatment
T21006 6285-6288 CC denotes and
T21005 6271-6284 JJ denotes heterogeneous
T21004 6259-6267 NNS denotes patients
T21003 6253-6258 DT denotes these
T21002 6250-6252 IN denotes of
T21001 6268-6270 VBZ denotes is
T21000 6240-6249 NN denotes phenotype
T20999 6236-6239 DT denotes The
T20998 6235-6367 sentence denotes The phenotype of these patients is heterogeneous and treatment with a wide range of thiamin doses has produced limited success [1].
T20997 6234-6235 . denotes .
T20996 6233-6234 -RRB- denotes ]
T20995 6232-6233 CD denotes 1
T20994 6231-6232 -LRB- denotes [
T20993 6216-6220 VBN denotes been
T20992 6212-6215 VBZ denotes has
T20991 6207-6211 NN denotes MSUD
T20990 6204-6206 IN denotes of
T20989 6221-6230 VBN denotes described
T20988 6187-6188 HYPH denotes -
T20987 6188-6198 JJ denotes responsive
T20986 6180-6187 NN denotes thiamin
T20985 6199-6203 NN denotes form
T20984 6178-6179 DT denotes A
T20983 6177-6235 sentence denotes A thiamin-responsive form of MSUD has been described [1].
T20982 6176-6177 . denotes .
T20981 6169-6176 NN denotes thiamin
T20980 6166-6168 IN denotes of
T20979 6159-6165 NN denotes effect
T20978 6155-6158 DT denotes the
T20977 6150-6154 VB denotes test
T20976 6147-6149 TO denotes to
T20975 6139-6146 NNS denotes studies
T20974 6135-6138 IN denotes for
T20973 6128-6134 JJ denotes useful
T20972 6120-6124 RB denotes also
T20971 6116-6119 MD denotes may
T20970 6105-6109 NN denotes MSUD
T20969 6092-6104 JJ denotes intermediate
T20968 6110-6115 NN denotes model
T20967 6088-6091 DT denotes the
T20966 6086-6088 , denotes ,
T20965 6125-6127 VB denotes be
T20964 6074-6086 RB denotes Additionally
T20963 6073-6177 sentence denotes Additionally, the intermediate MSUD model may also be useful for studies to test the effect of thiamin.
T20962 6072-6073 . denotes .
T20961 6066-6072 NN denotes tissue
T20960 6058-6065 NN denotes autopsy
T20959 6055-6057 IN denotes on
T20958 6045-6049 MD denotes must
T20957 6050-6054 VB denotes rely
T20956 6039-6044 RB denotes often
T20955 6035-6038 CC denotes and
T20954 6033-6035 , denotes ,
T20953 6018-6025 JJ denotes ethical
T20952 6026-6033 NNS denotes reasons
T20951 6010-6017 JJ denotes obvious
T20950 6006-6009 IN denotes for
T20949 5997-6005 NNS denotes patients
T20948 5991-5996 JJ denotes human
T20947 5988-5990 IN denotes in
T20946 5985-5987 VB denotes do
T20945 5982-5984 TO denotes to
T20944 5970-5971 HYPH denotes /
T20943 5971-5981 JJ denotes impossible
T20942 5961-5970 JJ denotes difficult
T20941 5956-5960 RB denotes very
T20940 5952-5955 VBP denotes are
T20939 5944-5951 NNS denotes studies
T20938 5939-5943 JJ denotes Such
T20937 5938-6073 sentence denotes Such studies are very difficult/impossible to do in human patients for obvious ethical reasons, and often must rely on autopsy tissue.
T20936 5937-5938 . denotes .
T20935 5930-5937 NN denotes disease
T20934 5926-5929 DT denotes the
T20933 5923-5925 IN denotes of
T20932 5893-5909 JJ denotes pathophysiologic
T20931 5882-5892 VBG denotes underlying
T20930 5910-5922 NNS denotes consequences
T20929 5878-5881 DT denotes the
T20928 5864-5877 VBG denotes investigating
T20927 5860-5863 IN denotes for
T20926 5853-5859 JJ denotes useful
T20925 5848-5852 RB denotes very
T20924 5840-5844 RB denotes also
T20923 5833-5839 MD denotes should
T20922 5826-5832 NNS denotes models
T20921 5820-5825 DT denotes these
T20920 5818-5820 , denotes ,
T20919 5808-5818 NNS denotes treatments
T20918 5800-5807 JJ denotes various
T20917 5792-5799 VBG denotes testing
T20916 5789-5791 IN denotes to
T20915 5780-5788 NN denotes addition
T20914 5845-5847 VB denotes be
T20913 5777-5779 IN denotes In
T20912 5776-5938 sentence denotes In addition to testing various treatments, these models should also be very useful for investigating the underlying pathophysiologic consequences of the disease.
T20911 5775-5776 . denotes .
T20910 5769-5775 NNS denotes humans
T20909 5766-5768 IN denotes in
T20908 5758-5765 NNS denotes studies
T20907 5754-5757 IN denotes for
T20906 5750-5753 NN denotes way
T20905 5746-5749 DT denotes the
T20904 5741-5745 VB denotes pave
T20903 5735-5740 MD denotes could
T20902 5731-5734 CC denotes and
T20901 5708-5719 JJ denotes therapeutic
T20900 5720-5730 NNS denotes approaches
T20899 5703-5707 JJ denotes such
T20898 5695-5702 VB denotes advance
T20897 5692-5694 TO denotes to
T20895 5672-5679 NNS denotes studies
T20894 5665-5671 NN denotes animal
T20893 5660-5664 IN denotes with
T20892 5683-5691 VBN denotes expected
T20891 5652-5659 NN denotes Success
T20890 5651-5776 sentence denotes Success with animal studies is expected to advance such therapeutic approaches and could pave the way for studies in humans.
T20889 5650-5651 . denotes .
T20888 5644-5650 NN denotes safety
T20887 5638-5639 HYPH denotes -
T20886 5634-5638 JJ denotes long
T20885 5630-5633 CC denotes and
T20884 5628-5629 HYPH denotes -
T20883 5639-5643 NN denotes term
T20882 5623-5628 JJ denotes short
T20881 5618-5622 RB denotes also
T20880 5614-5617 CC denotes but
T20879 5612-5614 , denotes ,
T20878 5600-5603 DT denotes the
T20877 5595-5599 RB denotes only
T20876 5604-5612 NN denotes efficacy
T20875 5591-5594 RB denotes not
T20874 5578-5590 VBG denotes establishing
T20873 5575-5577 IN denotes of
T20872 5556-5567 JJ denotes preliminary
T20871 5568-5574 NN denotes method
T20870 5554-5555 DT denotes a
T20869 5531-5536 NN denotes model
T20868 5524-5530 NN denotes animal
T20867 5537-5544 NNS denotes systems
T20866 5512-5523 JJ denotes appropriate
T20865 5509-5511 IN denotes on
T20864 5499-5508 NNS denotes therapies
T20863 5488-5492 NN denotes cell
T20862 5484-5487 CC denotes and
T20861 5493-5498 VBN denotes based
T20860 5479-5483 NN denotes gene
T20859 5476-5478 IN denotes of
T20858 5545-5553 VBZ denotes provides
T20857 5468-5475 NN denotes testing
T20856 5464-5467 DT denotes The
T20855 5463-5651 sentence denotes The testing of gene and cell based therapies on appropriate animal model systems provides a preliminary method of establishing not only the efficacy, but also short- and long-term safety.
T20854 5462-5463 . denotes .
T20853 5455-5462 NNS denotes studies
T20852 5443-5454 JJ denotes preclinical
T20851 5439-5442 IN denotes for
T20850 5430-5438 NNS denotes diseases
T20849 5422-5429 JJ denotes genetic
T20848 5419-5421 IN denotes of
T20847 5412-5418 NNS denotes models
T20846 5405-5411 NN denotes animal
T20845 5402-5404 IN denotes of
T20844 5397-5401 NN denotes need
T20843 5388-5396 JJ denotes critical
T20842 5384-5387 CC denotes and
T20841 5373-5383 NN denotes importance
T20840 5369-5372 DT denotes the
T20839 5352-5358 NNS denotes humans
T21116 6831-6832 -RRB- denotes ]
T21115 6828-6829 , denotes ,
T21114 6826-6828 CD denotes 45
T21113 6825-6826 , denotes ,
T20838 5349-5351 IN denotes in
T20837 5341-5348 NN denotes therapy
T20836 5336-5340 NN denotes gene
T20835 5331-5335 IN denotes with
T20834 5322-5330 NNS denotes problems
T20833 5315-5321 JJ denotes recent
T20832 5313-5315 , denotes ,
T20831 5306-5313 NN denotes therapy
T20830 5301-5305 NN denotes gene
T20829 5298-5300 IN denotes to
T20828 5359-5368 VBP denotes highlight
T20827 5289-5297 JJ denotes Relevant
T20826 5288-5463 sentence denotes Relevant to gene therapy, recent problems with gene therapy in humans highlight the importance and critical need of animal models of genetic diseases for preclinical studies.
T20825 5287-5288 . denotes .
T20824 5286-5287 -RRB- denotes )
T20823 5285-5286 -RRB- denotes ]
T20822 5283-5285 CD denotes 44
T20821 5282-5283 -LRB- denotes [
T20820 5271-5275 NN denotes stem
T20819 5276-5281 NNS denotes cells
T20818 5261-5270 JJ denotes embryonic
T20817 5258-5260 CC denotes or
T20816 5256-5257 -RRB- denotes ]
T20815 5253-5254 , denotes ,
T20814 5251-5253 CD denotes 42
T20813 5254-5256 CD denotes 43
T20812 5250-5251 -LRB- denotes [
T20811 5236-5238 , denotes ,
T20810 5232-5236 FW denotes e.g.
T20809 5238-5249 NNS denotes hepatocytes
T20808 5231-5232 -LRB- denotes (
T20807 5221-5230 NNS denotes therapies
T20806 5210-5214 NN denotes cell
T20805 5206-5209 CC denotes and
T20804 5204-5205 -RRB- denotes ]
T20803 5202-5204 CD denotes 41
T20802 5201-5202 SYM denotes -
T20801 5199-5201 CD denotes 38
T20800 5198-5199 -LRB- denotes [
T20799 5215-5220 VBN denotes based
T20798 5193-5197 NN denotes gene
T20797 5190-5192 IN denotes as
T20796 5185-5189 JJ denotes such
T20795 5174-5184 NNS denotes treatments
T20794 5168-5173 JJ denotes novel
T20793 5163-5167 VB denotes test
T20792 5160-5162 TO denotes to
T20791 5144-5149 NN denotes mouse
T20790 5139-5143 NN denotes MSUD
T20789 5150-5156 NNS denotes models
T20788 5135-5138 DT denotes the
T20787 5132-5134 IN denotes by
T20786 5122-5131 VBN denotes presented
T20785 5157-5159 VBZ denotes is
T20784 5098-5109 JJ denotes significant
T20783 5093-5097 RBS denotes most
T20782 5110-5121 NN denotes opportunity
T20781 5089-5092 DT denotes The
T20780 5088-5288 sentence denotes The most significant opportunity presented by the MSUD mouse models is to test novel treatments such as gene [38-41] and cell based therapies (e.g., hepatocytes [42,43] or embryonic stem cells [44]).
T20779 5087-5088 . denotes .
T20778 5077-5082 JJ denotes human
T20777 5083-5087 NN denotes MSUD
T20776 5068-5076 VBG denotes modeling
T20775 5064-5067 IN denotes for
T20774 5052-5063 JJ denotes appropriate
T20773 5048-5051 CC denotes and
T20772 5042-5047 JJ denotes valid
T20771 5037-5041 RBR denotes more
T20770 5026-5032 NN denotes report
T20769 5021-5025 DT denotes this
T20768 5018-5020 IN denotes in
T20767 5008-5017 VBN denotes described
T20766 4994-5000 JJ denotes murine
T20765 5001-5007 NNS denotes models
T20764 4990-4993 DT denotes the
T20763 4988-4990 , denotes ,
T20762 4980-4988 NN denotes contrast
T20761 5033-5036 VBP denotes are
T20760 4977-4979 IN denotes In
T20759 4976-5088 sentence denotes In contrast, the murine models described in this report are more valid and appropriate for modeling human MSUD.
T20758 4975-4976 . denotes .
T20757 4968-4975 NN denotes utility
T20756 4960-4967 JJ denotes limited
T20755 4957-4959 IN denotes of
T20754 4952-4956 RB denotes also
T20753 4943-4948 NN denotes model
T20752 4938-4942 DT denotes this
T20751 4936-4938 , denotes ,
T20750 4935-4936 -RRB- denotes ]
T20749 4934-4935 CD denotes 1
T20748 4933-4934 -LRB- denotes [
T20747 4926-4932 NNS denotes humans
T20746 4921-4925 NN denotes MSUD
T20745 4917-4920 CC denotes and
T20744 4907-4910 NN denotes cow
T20743 4902-4906 NN denotes MSUD
T20742 4911-4916 NN denotes model
T20741 4898-4901 DT denotes the
T20740 4890-4897 IN denotes between
T20739 4878-4889 NNS denotes differences
T20738 4875-4877 IN denotes of
T20737 4863-4874 NN denotes observation
T20736 4859-4862 DT denotes the
T20735 4856-4858 IN denotes to
T20734 4852-4855 IN denotes due
T20733 4848-4851 CC denotes and
T20732 4835-4841 NN denotes animal
T20731 4829-4834 JJ denotes large
T20730 4827-4828 DT denotes a
T20729 4842-4847 NN denotes model
T20728 4822-4826 JJ denotes such
T20727 4819-4821 IN denotes by
T20726 4811-4818 VBN denotes imposed
T20725 4799-4810 NNS denotes constraints
T20724 4789-4798 JJ denotes practical
T20723 4786-4788 IN denotes to
T20722 4782-4785 IN denotes due
T20721 4780-4782 , denotes ,
T20720 4949-4951 VBZ denotes is
T20719 4773-4780 RB denotes However
T20718 4772-4976 sentence denotes However, due to practical constraints imposed by such a large animal model and due to the observation of differences between the MSUD cow model and MSUD humans [1], this model is also of limited utility.
T20717 4771-4772 . denotes .
T20716 4770-4771 -RRB- denotes ]
T20715 4768-4770 CD denotes 19
T20714 4767-4768 SYM denotes -
T20713 4765-4767 CD denotes 17
T20712 4764-4765 -LRB- denotes [
T20711 4743-4753 RB denotes previously
T20710 4738-4742 VBN denotes been
T20709 4733-4737 RB denotes also
T20708 4729-4732 VBZ denotes has
T20707 4724-4728 NN denotes MSUD
T20706 4721-4723 IN denotes of
T20705 4711-4714 NN denotes cow
T20704 4715-4720 NN denotes model
T20703 4709-4710 DT denotes a
T20702 4707-4709 , denotes ,
T20701 4696-4701 NN denotes mouse
T20700 4702-4707 NN denotes model
T20699 4691-4695 DT denotes this
T20698 4688-4690 IN denotes to
T20697 4679-4687 NN denotes addition
T20696 4754-4763 VBN denotes described
T20695 4676-4678 IN denotes In
T20694 4675-4772 sentence denotes In addition to this mouse model, a cow model of MSUD has also been previously described [17-19].
T20693 4674-4675 . denotes .
T20692 4664-4674 NN denotes deficiency
T20691 4659-4663 NN denotes BCAT
T20690 4656-4658 IN denotes to
T20689 4648-4655 NN denotes disease
T20688 4643-4647 DT denotes this
T20687 4631-4642 VBG denotes attributing
T20686 4616-4620 VBN denotes been
T20685 4612-4615 VBZ denotes has
T20684 4607-4611 NN denotes MSUD
T20683 4604-4606 IN denotes of
T20682 4599-4603 NN denotes case
T20681 4596-4598 DT denotes no
T20680 4594-4596 , denotes ,
T20674 4565-4570 NN denotes BCKDH
T20673 4562-4564 IN denotes of
T20672 4571-4574 VBP denotes are
T20671 4555-4561 NNS denotes levels
T20670 4551-4554 DT denotes the
T20669 4547-4550 CC denotes and
T20668 4541-4546 NN denotes BCKDH
T20667 4538-4540 IN denotes in
T20666 4534-4537 RB denotes not
T20665 4522-4530 NN denotes mutation
T20664 4518-4521 DT denotes the
T20663 4531-4533 VBZ denotes is
T20662 4510-4517 IN denotes because
T20661 4505-4509 NN denotes MSUD
T20660 4502-4504 IN denotes of
T20659 4491-4495 JJ denotes true
T20658 4496-4501 NN denotes model
T20657 4489-4490 DT denotes a
T20656 4485-4488 RB denotes not
T20655 4479-4481 PRP denotes it
T20654 4477-4479 , denotes ,
T20653 4460-4471 JJ denotes interesting
T20652 4472-4477 NN denotes model
T20651 4457-4459 DT denotes an
T20650 4482-4484 VBZ denotes is
T20649 4449-4453 DT denotes this
T20648 4454-4456 VBZ denotes is
T20647 4443-4448 IN denotes While
T20646 4442-4582 sentence denotes While this is an interesting model, it is not a true model of MSUD because the mutation is not in BCKDH and the levels of BCKDH are normal.
T20645 4441-4442 . denotes .
T20644 4433-4441 JJ denotes elevated
T20643 4424-4432 RB denotes markedly
T20642 4407-4411 NN denotes BCAA
T20641 4401-4406 NN denotes blood
T20640 4412-4418 NNS denotes levels
T20639 4397-4400 DT denotes the
T20638 4390-4396 IN denotes though
T20637 4419-4423 VBD denotes were
T20636 4385-4389 RB denotes even
T20635 4378-4384 JJ denotes normal
T20634 4368-4373 NN denotes mouse
T20633 4363-4367 DT denotes this
T20632 4360-4362 IN denotes of
T20631 4353-4359 NN denotes muscle
T20630 4349-4352 CC denotes and
T20629 4343-4348 NN denotes liver
T20628 4339-4342 DT denotes the
T20627 4336-4338 IN denotes in
T20626 4374-4377 VBD denotes was
T20625 4321-4326 NN denotes BCKDH
T20624 4327-4335 NN denotes activity
T20623 4317-4320 DT denotes The
T20622 4316-4442 sentence denotes The BCKDH activity in the liver and muscle of this mouse was normal even though the blood BCAA levels were markedly elevated.
T20621 4315-4316 . denotes .
T20620 4314-4315 -RRB- denotes ]
T20619 4312-4314 CD denotes 20
T20618 4311-4312 -LRB- denotes [
T20896 5680-5682 VBZ denotes is
T20617 4301-4305 NN denotes BCAT
T20616 4287-4300 JJ denotes mitochondrial
T20615 4306-4310 NN denotes gene
T20614 4283-4286 DT denotes the
T20613 4272-4282 VBG denotes disrupting
T20612 4269-4271 IN denotes by
T20611 4250-4260 RB denotes previously
T20610 4246-4249 VBD denotes was
T20609 4241-4245 NN denotes MSUD
T20608 4235-4240 JJ denotes human
T20607 4224-4234 VBG denotes resembling
T20606 4261-4268 VBN denotes created
T20605 4214-4217 NN denotes ENU
T20604 4218-4223 NN denotes mouse
T20603 4211-4213 DT denotes An
T20602 4210-4316 sentence denotes An ENU mouse resembling human MSUD was previously created by disrupting the mitochondrial BCAT gene [20].
T20601 4209-4210 . denotes .
T20600 4193-4202 VBN denotes described
T20599 4182-4192 RB denotes previously
T20598 4203-4209 NNS denotes models
T20597 4178-4181 DT denotes the
T20596 4173-4177 IN denotes over
T20595 4164-4172 NNS denotes advances
T20594 4154-4163 JJ denotes important
T20593 4136-4149 NN denotes communication
T20592 4131-4135 DT denotes this
T20591 4128-4130 IN denotes in
T20590 4118-4127 VBN denotes described
T20589 4150-4153 VBP denotes are
T20588 4111-4117 NNS denotes models
T20587 4107-4110 DT denotes The
T20586 4106-4210 sentence denotes The models described in this communication are important advances over the previously described models.
T20585 4105-4106 . denotes .
T20584 4093-4095 VB denotes be
T20583 4088-4092 RB denotes also
T20582 4096-4105 VBN denotes regulated
T20581 4084-4087 MD denotes can
T20580 4080-4083 CC denotes and
T20579 4071-4079 NN denotes survival
T20578 4067-4070 IN denotes for
T20577 4061-4066 VBP denotes allow
T20576 4056-4060 WDT denotes that
T20575 4050-4055 NNS denotes lines
T20574 4045-4049 VB denotes find
T20573 4042-4044 TO denotes to
T20572 4032-4034 VB denotes be
T20571 4028-4031 MD denotes may
T20570 4022-4027 NNS denotes lines
T20569 4011-4021 JJ denotes additional
T20568 4008-4010 IN denotes of
T20567 4035-4041 VBN denotes needed
T20566 3998-4007 NN denotes Screening
T20565 3997-4106 sentence denotes Screening of additional lines may be needed to find lines that allow for survival and can also be regulated.
T20564 3996-3997 . denotes .
T20563 3986-3996 NN denotes expression
T20562 3976-3985 VBN denotes regulated
T20561 3972-3975 IN denotes for
T20560 3961-3971 JJ denotes permissive
T20559 3957-3960 RB denotes not
T20558 3942-3952 NNS denotes transgenes
T20557 3938-3941 DT denotes the
T20556 3935-3937 IN denotes of
T20555 3918-3929 NN denotes integration
T20554 3930-3934 NN denotes site
T20553 3914-3917 DT denotes the
T20552 3953-3956 VBD denotes was
T20551 3909-3913 IN denotes that
T20550 3897-3908 JJ denotes conceivable
T20549 3894-3896 VBZ denotes is
T20548 3891-3893 PRP denotes It
T20547 3890-3997 sentence denotes It is conceivable that the integration site of the transgenes was not permissive for regulated expression.
T20546 3889-3890 . denotes .
T20545 3882-3889 JJ denotes unknown
T20544 3867-3878 NN denotes doxycycline
T20543 3864-3866 IN denotes to
T20542 3851-3863 JJ denotes unresponsive
T20541 3846-3850 VBD denotes were
T20540 3836-3839 CD denotes two
T20539 3840-3845 NNS denotes lines
T20538 3830-3835 DT denotes these
T20537 3879-3881 VBZ denotes is
T20536 3823-3829 NN denotes reason
T20535 3819-3822 DT denotes The
T20534 3818-3890 sentence denotes The reason these two lines were unresponsive to doxycycline is unknown.
T20533 3817-3818 . denotes .
T20532 3816-3817 -RRB- denotes )
T20531 3807-3810 RB denotes not
T20530 3802-3806 NNS denotes data
T20529 3811-3816 VBN denotes shown
T20528 3801-3802 -LRB- denotes (
T20527 3794-3800 VBN denotes tested
T20526 3777-3787 JJ denotes transgenic
T20525 3773-3776 CD denotes two
T20524 3788-3793 NNS denotes lines
T20523 3769-3772 DT denotes the
T20522 3766-3768 IN denotes of
T20521 3759-3765 DT denotes either
T20520 3756-3758 IN denotes in
T20519 3744-3755 NN denotes doxycycline
T20518 3742-3744 , denotes ,
T20517 3721-3733 NN denotes tetracycline
T20516 3714-3720 JJ denotes potent
T20515 3734-3742 NN denotes analogue
T20514 3710-3713 DT denotes the
T20513 3707-3709 IN denotes by
T20512 3687-3697 NN denotes expression
T20511 3684-3686 NN denotes E2
T20510 3678-3683 JJ denotes human
T20509 3698-3706 NN denotes cassette
T20508 3674-3677 DT denotes the
T20507 3670-3673 RP denotes off
T20506 3652-3664 RB denotes consistently
T20505 3665-3669 VB denotes turn
T20504 3649-3651 TO denotes to
T20503 3642-3648 JJ denotes unable
T20502 3634-3636 PRP denotes we
T20501 3632-3634 , denotes ,
T20500 3637-3641 VBD denotes were
T20499 3625-3632 RB denotes However
T20498 3624-3818 sentence denotes However, we were unable to consistently turn off the human E2 expression cassette by the potent tetracycline analogue, doxycycline in either of the two transgenic lines tested (data not shown).
T20497 3623-3624 . denotes .
T20496 3605-3613 VBG denotes rescuing
T20495 3614-3623 NN denotes transgene
T20494 3601-3604 DT denotes the
T20493 3597-3600 RP denotes off
T20492 3589-3596 VBG denotes turning
T20491 3586-3588 IN denotes by
T20490 3572-3574 POS denotes 's
T20489 3575-3585 NN denotes discretion
T20488 3560-3572 NN denotes investigator
T20487 3556-3559 DT denotes the
T20486 3553-3555 IN denotes at
T20485 3535-3542 JJ denotes classic
T20484 3543-3552 NN denotes phenotype
T20483 3531-3534 DT denotes the
T20482 3528-3530 IN denotes to
T20481 3515-3517 VB denotes be
T20480 3509-3514 MD denotes could
T20479 3518-3527 VBN denotes converted
T20478 3504-3508 WDT denotes that
T20477 3489-3493 NN denotes MSUD
T20476 3476-3488 JJ denotes intermediate
T20475 3494-3503 NN denotes phenotype
T20474 3473-3475 DT denotes an
T20473 3468-3472 IN denotes with
T20472 3463-3467 NNS denotes mice
T20471 3456-3462 VB denotes create
T20470 3453-3455 TO denotes to
T20469 3440-3448 NN denotes approach
T20468 3436-3439 PRP$ denotes our
T20467 3429-3435 IN denotes behind
T20466 3449-3452 VBD denotes was
T20465 3420-3428 NN denotes strategy
T20464 3416-3419 DT denotes The
T20463 3415-3624 sentence denotes The strategy behind our approach was to create mice with an intermediate MSUD phenotype that could be converted to the classic phenotype at the investigator's discretion by turning off the rescuing transgene.
T20462 3414-3415 . denotes .
T20461 3413-3414 -RRB- denotes ]
T20460 3411-3413 CD denotes 34
T20459 3410-3411 -LRB- denotes [
T20448 3347-3350 VBZ denotes has
T20447 3356-3360 VBN denotes used
T20446 3342-3346 WDT denotes that
T20445 3328-3334 NN denotes switch
T20444 3323-3327 NN denotes gene
T20443 3313-3322 VBN denotes regulated
T20442 3300-3312 NN denotes tetracycline
T20441 3335-3341 NN denotes system
T20440 3296-3299 DT denotes the
T20439 3293-3295 IN denotes on
T20438 3283-3286 VBD denotes was
T20437 3272-3276 NN denotes MSUD
T20436 3259-3271 JJ denotes intermediate
T20435 3277-3282 NN denotes model
T20434 3255-3258 DT denotes the
T20433 3248-3254 VB denotes create
T20432 3245-3247 TO denotes to
T20431 3236-3239 VBD denotes was
T20430 3240-3244 VBN denotes used
T20429 3231-3235 WDT denotes that
T20428 3287-3292 VBN denotes based
T20427 3211-3221 JJ denotes transgenic
T20426 3222-3230 NN denotes approach
T20425 3207-3210 DT denotes The
T20424 3206-3415 sentence denotes The transgenic approach that was used to create the intermediate MSUD model was based on the tetracycline regulated gene switch system that has been used with great success in other studies, for example [34].
T20423 3205-3206 . denotes .
T20422 3201-3205 NN denotes MSUD
T20421 3198-3200 IN denotes of
T20420 3180-3192 JJ denotes intermediate
T20419 3193-3197 NN denotes form
T20418 3176-3179 DT denotes the
T20417 3173-3175 IN denotes of
T20416 3160-3166 NN denotes animal
T20415 3154-3159 JJ denotes small
T20414 3147-3153 JJ denotes useful
T20413 3167-3172 NN denotes model
T20412 3145-3146 DT denotes a
T20411 3119-3129 VBN denotes engineered
T20410 3107-3118 RB denotes genetically
T20409 3130-3134 NNS denotes mice
T20408 3101-3106 DT denotes these
T20407 3099-3101 , denotes ,
T20406 3085-3090 JJ denotes human
T20405 3091-3099 NNS denotes patients
T20404 3081-3084 DT denotes the
T20403 3078-3080 IN denotes to
T20402 3066-3072 NN denotes rescue
T20401 3073-3077 NNS denotes mice
T20400 3062-3065 DT denotes the
T20399 3059-3061 IN denotes of
T20398 3035-3045 JJ denotes phenotypic
T20397 3046-3058 NNS denotes similarities
T20396 3031-3034 DT denotes the
T20395 3028-3030 IN denotes of
T20394 3135-3144 VBP denotes represent
T20393 3020-3027 IN denotes Because
T20392 3019-3206 sentence denotes Because of the phenotypic similarities of the rescue mice to the human patients, these genetically engineered mice represent a useful small animal model of the intermediate form of MSUD.
T20679 4621-4630 VBN denotes described
T20678 4583-4594 RB denotes Furthermore
T20677 4582-4675 sentence denotes Furthermore, no case of MSUD has been described attributing this disease to BCAT deficiency.
T20676 4581-4582 . denotes .
T20675 4575-4581 JJ denotes normal
T20391 3018-3019 . denotes .
T20390 3017-3018 -RRB- denotes ]
T20389 3016-3017 CD denotes 1
T20388 3015-3016 -LRB- denotes [
T20387 3008-3014 JJ denotes normal
T20386 3005-3007 IN denotes of
T20385 3003-3004 NN denotes %
T20384 3000-3001 SYM denotes –
T20383 3001-3003 CD denotes 30
T20382 2999-3000 CD denotes 3
T20381 2996-2998 IN denotes of
T20380 2990-2995 NN denotes range
T20379 2986-2989 DT denotes the
T20378 2983-2985 IN denotes in
T20377 2968-2973 NN denotes BCKDH
T20376 2964-2967 CC denotes and
T20375 2974-2982 NN denotes activity
T20374 2959-2963 NN denotes BCAA
T20373 2956-2958 IN denotes of
T20372 2949-2955 NNS denotes levels
T20371 2939-2948 VBN denotes increased
T20370 2926-2938 RB denotes persistently
T20369 2907-2912 JJ denotes human
T20368 2902-2906 NN denotes MSUD
T20367 2913-2921 NNS denotes patients
T20366 2889-2901 JJ denotes intermediate
T20365 2886-2888 IN denotes of
T20364 2922-2925 VBP denotes are
T20363 2876-2885 NNS denotes hallmarks
T20362 2872-2875 DT denotes The
T20361 2871-3019 sentence denotes The hallmarks of intermediate MSUD human patients are persistently increased levels of BCAA and BCKDH activity in the range of 3–30% of normal [1].
T20360 2870-2871 . denotes .
T20359 2861-2870 NNS denotes knockouts
T20358 2857-2860 CC denotes and
T20357 2848-2856 NNS denotes controls
T20356 2840-2847 IN denotes between
T20355 2827-2839 JJ denotes intermediate
T20354 2810-2816 NN denotes rescue
T20353 2817-2821 NNS denotes mice
T20352 2804-2809 DT denotes these
T20351 2801-2803 IN denotes of
T20350 2795-2800 NN denotes blood
T20349 2792-2794 IN denotes in
T20348 2785-2791 NNS denotes levels
T20347 2780-2784 NN denotes BCAA
T20346 2822-2826 VBD denotes were
T20345 2775-2779 IN denotes that
T20344 2757-2761 RB denotes also
T20343 2762-2774 VBD denotes demonstrated
T20342 2754-2756 PRP denotes We
T20341 2753-2871 sentence denotes We also demonstrated that BCAA levels in blood of these rescue mice were intermediate between controls and knockouts.
T20340 2752-2753 . denotes .
T20339 2744-2752 NN denotes survival
T20338 2738-2743 VB denotes allow
T20337 2735-2737 TO denotes to
T20336 2724-2734 JJ denotes sufficient
T20335 2714-2719 NN denotes liver
T20334 2710-2713 DT denotes the
T20333 2707-2709 IN denotes in
T20332 2692-2697 NN denotes BCKDH
T20331 2698-2706 NN denotes activity
T20330 2685-2691 JJ denotes normal
T20329 2682-2684 IN denotes of
T20328 2680-2681 NN denotes %
T20327 2678-2679 SYM denotes –
T20326 2677-2678 CD denotes 5
T20325 2676-2677 SYM denotes ~
T20324 2679-2680 CD denotes 6
T20323 2671-2675 RB denotes only
T20322 2659-2664 NN denotes mouse
T20321 2665-2670 NNS denotes lines
T20320 2653-2658 DT denotes these
T20319 2650-2652 IN denotes in
T20318 2720-2723 VBD denotes was
T20317 2645-2649 IN denotes that
T20316 2640-2644 VB denotes note
T20315 2637-2639 TO denotes to
T20314 2625-2636 JJ denotes interesting
T20313 2622-2624 VBZ denotes is
T20312 2619-2621 PRP denotes It
T20311 2618-2753 sentence denotes It is interesting to note that in these mouse lines only ~5–6% of normal BCKDH activity in the liver was sufficient to allow survival.
T20310 2617-2618 . denotes .
T20309 2608-2617 NN denotes adulthood
T20308 2605-2607 IN denotes to
T20307 2584-2590 NN denotes rescue
T20306 2591-2595 NNS denotes mice
T20305 2580-2583 DT denotes the
T20304 2577-2579 IN denotes of
T20303 2596-2604 VBD denotes survived
T20302 2572-2576 JJ denotes Many
T20301 2571-2618 sentence denotes Many of the rescue mice survived to adulthood.
T20300 2570-2571 . denotes .
T20299 2552-2560 JJ denotes neonatal
T20298 2561-2570 NN denotes lethality
T20297 2548-2551 DT denotes the
T20296 2541-2547 VB denotes rescue
T20295 2538-2540 TO denotes to
T20294 2533-2537 JJ denotes able
T20293 2516-2524 NN denotes knockout
T20292 2525-2529 NNS denotes mice
T20291 2510-2515 DT denotes these
T20290 2507-2509 IN denotes of
T20289 2501-2506 NN denotes liver
T20288 2497-2500 DT denotes the
T20287 2494-2496 IN denotes in
T20286 2481-2483 NN denotes E2
T20285 2475-2480 JJ denotes human
T20284 2484-2493 NN denotes transgene
T20283 2473-2474 DT denotes a
T20282 2470-2472 IN denotes of
T20281 2459-2469 NN denotes expression
T20280 2530-2532 VBZ denotes is
T20279 2454-2458 IN denotes that
T20278 2449-2453 VBP denotes show
T20277 2446-2448 PRP denotes We
T20276 2445-2571 sentence denotes We show that expression of a human E2 transgene in the liver of these knockout mice is able to rescue the neonatal lethality.
T20275 2444-2445 . denotes .
T20274 2429-2437 JJ denotes neonatal
T20273 2423-2428 JJ denotes early
T20272 2438-2444 NN denotes period
T20271 2419-2422 DT denotes the
T20270 2412-2418 IN denotes during
T20269 2405-2407 NN denotes E2
T20268 2397-2404 VBN denotes derived
T20267 2387-2396 NN denotes transgene
T20266 2379-2386 IN denotes without
T20265 2369-2378 NNS denotes knockouts
T20264 2366-2368 NN denotes E2
T20263 2364-2366 , denotes ,
T20262 2359-2364 RB denotes above
T20261 2408-2411 VBP denotes die
T20260 2349-2358 VBN denotes mentioned
T20259 2346-2348 IN denotes As
T20258 2345-2445 sentence denotes As mentioned above, E2 knockouts without transgene derived E2 die during the early neonatal period.
T20257 2344-2345 . denotes .
T20256 2331-2339 NN denotes knockout
T20255 2340-2344 NNS denotes mice
T20254 2328-2330 NN denotes E2
T20253 2325-2327 IN denotes of
T20252 2319-2324 NN denotes liver
T20251 2315-2318 DT denotes the
T20250 2312-2314 IN denotes in
T20249 2309-2311 NN denotes E2
T20248 2303-2308 JJ denotes human
T20247 2295-2302 VB denotes express
T20246 2292-2294 TO denotes to
T20245 2272-2282 JJ denotes transgenic
T20244 2283-2291 NN denotes strategy
T20243 2270-2271 DT denotes a
T20242 2262-2264 PRP denotes we
T20241 2260-2262 , denotes ,
T20240 2256-2260 NN denotes MSUD
T20239 2243-2255 JJ denotes intermediate
T20238 2240-2242 IN denotes of
T20237 2234-2239 NN denotes model
T20236 2232-2233 DT denotes a
T20235 2265-2269 VBD denotes used
T20234 2225-2231 VB denotes create
T20233 2222-2224 TO denotes To
T20227 2194-2205 JJ denotes biochemical
T20226 2206-2216 NNS denotes phenotypes
T20225 2186-2193 JJ denotes several
T20224 2183-2185 IN denotes to
T20223 2175-2182 NN denotes respect
T20222 2170-2174 IN denotes with
T20221 2156-2161 JJ denotes human
T20220 2162-2169 NN denotes disease
T20219 2152-2155 DT denotes the
T20218 2149-2151 IN denotes of
T20217 2134-2142 JJ denotes faithful
T20216 2143-2148 NN denotes model
T20215 2132-2133 DT denotes a
T20214 2129-2131 VB denotes be
T20213 2126-2128 TO denotes to
T20212 2118-2125 VBZ denotes appears
T20211 2114-2117 CC denotes and
T20210 2109-2113 NN denotes MSUD
T20209 2101-2108 JJ denotes classic
T20208 2098-2100 IN denotes of
T20207 2092-2097 NN denotes model
T20206 2090-2091 DT denotes a
T20205 2068-2078 RB denotes accurately
T20204 2053-2061 NN denotes mutation
T20203 2048-2052 JJ denotes null
T20202 2062-2067 NN denotes mouse
T20201 2044-2047 DT denotes the
T20200 2042-2044 , denotes ,
T20199 2039-2042 RB denotes far
T20198 2034-2038 RB denotes thus
T20197 2024-2033 VBN denotes completed
T20196 1999-2015 NN denotes characterization
T20195 2016-2023 NNS denotes studies
T20194 1995-1998 DT denotes the
T20193 2079-2089 VBZ denotes represents
T20192 1990-1994 IN denotes From
T20191 1989-2221 sentence denotes From the characterization studies completed thus far, the null mutation mouse accurately represents a model of classic MSUD and appears to be a faithful model of the human disease with respect to several biochemical phenotypes [1].
T20190 1988-1989 . denotes .
T20189 1970-1979 JJ denotes enzymatic
T20188 1980-1988 NN denotes activity
T20187 1964-1969 NN denotes BCKDH
T20186 1961-1963 IN denotes of
T20185 1956-1960 NN denotes half
T20184 1942-1955 RB denotes approximately
T20183 1935-1941 VBG denotes having
T20182 1927-1934 IN denotes despite
T20181 1922-1926 NN denotes BCAA
T20180 1919-1921 IN denotes of
T20179 1912-1918 NNS denotes levels
T20178 1905-1911 JJ denotes normal
T20177 1901-1904 VBD denotes had
T20176 1897-1900 CC denotes and
T20175 1890-1896 JJ denotes normal
T20174 1885-1889 VBD denotes were
T20173 1875-1884 NNS denotes knockouts
T20172 1862-1874 JJ denotes Heterozygous
T20171 1861-1989 sentence denotes Heterozygous knockouts were normal and had normal levels of BCAA despite having approximately half of BCKDH enzymatic activity.
T20458 3402-3409 NN denotes example
T20457 3398-3401 IN denotes for
T20456 3396-3398 , denotes ,
T20455 3389-3396 NNS denotes studies
T20454 3383-3388 JJ denotes other
T20453 3380-3382 IN denotes in
T20452 3372-3379 NN denotes success
T20451 3366-3371 JJ denotes great
T20450 3361-3365 IN denotes with
T20449 3351-3355 VBN denotes been
T20170 1860-1861 . denotes .
T20169 1859-1860 -RRB- denotes ]
T20168 1858-1859 CD denotes 1
T20167 1857-1858 -LRB- denotes [
T20166 1843-1847 NN denotes MSUD
T20165 1848-1856 NNS denotes patients
T20164 1835-1842 JJ denotes classic
T20163 1832-1834 IN denotes in
T20162 1828-1831 CC denotes and
T20161 1826-1827 -RRB- denotes ]
T20160 1824-1826 CD denotes 17
T20159 1823-1824 -LRB- denotes [
T20158 1813-1817 NN denotes MSUD
T20157 1818-1822 NN denotes calf
T20156 1809-1812 DT denotes the
T20155 1806-1808 IN denotes in
T20154 1797-1805 VBN denotes observed
T20153 1794-1796 IN denotes as
T20152 1781-1793 NN denotes malnutrition
T20151 1777-1780 CC denotes and
T20150 1775-1777 , denotes ,
T20149 1764-1775 NN denotes dehydration
T20148 1762-1764 , denotes ,
T20147 1757-1762 NN denotes edema
T20146 1751-1756 NN denotes brain
T20145 1749-1751 , denotes ,
T20144 1737-1749 NN denotes ketoacidosis
T20143 1735-1737 , denotes ,
T20142 1729-1735 NNS denotes levels
T20141 1718-1728 JJ denotes neurotoxic
T20140 1715-1717 IN denotes to
T20139 1710-1714 NN denotes BCAA
T20138 1707-1709 IN denotes of
T20137 1694-1706 NN denotes accumulation
T20136 1690-1693 DT denotes the
T20135 1687-1689 IN denotes to
T20134 1683-1686 IN denotes due
T20133 1676-1682 RB denotes likely
T20132 1673-1675 VBZ denotes is
T20131 1654-1662 JJ denotes neonatal
T20130 1663-1672 NN denotes lethality
T20129 1649-1653 DT denotes This
T20128 1648-1861 sentence denotes This neonatal lethality is likely due to the accumulation of BCAA to neurotoxic levels, ketoacidosis, brain edema, dehydration, and malnutrition as observed in the MSUD calf [17] and in classic MSUD patients [1].
T20127 1647-1648 . denotes .
T20126 1642-1647 NN denotes birth
T20125 1639-1641 IN denotes of
T20124 1633-1638 NNS denotes hours
T20123 1630-1632 CD denotes 72
T20122 1623-1629 IN denotes within
T20121 1602-1612 JJ denotes homozygous
T20120 1613-1617 NNS denotes pups
T20119 1598-1601 DT denotes the
T20118 1595-1597 IN denotes of
T20117 1591-1594 DT denotes all
T20116 1584-1590 RB denotes nearly
T20115 1582-1584 , denotes ,
T20114 1618-1622 VBD denotes died
T20113 1575-1582 RB denotes Finally
T20112 1574-1648 sentence denotes Finally, nearly all of the homozygous pups died within 72 hours of birth.
T20111 1573-1574 . denotes .
T20110 1572-1573 -RRB- denotes ]
T20109 1569-1570 , denotes ,
T20108 1568-1569 CD denotes 1
T20107 1570-1572 CD denotes 37
T20106 1567-1568 -LRB- denotes [
T20105 1558-1566 NNS denotes patients
T20104 1555-1557 IN denotes in
T20103 1538-1554 NNS denotes encephalopathies
T20102 1533-1537 NN denotes MSUD
T20101 1529-1532 IN denotes for
T20100 1521-1528 NN denotes culprit
T20099 1517-1520 DT denotes the
T20098 1502-1505 VBP denotes are
T20097 1506-1516 VBN denotes considered
T20096 1496-1501 WDT denotes which
T20095 1494-1496 , denotes ,
T20094 1483-1488 NN denotes amino
T20093 1471-1482 JJ denotes neuroactive
T20092 1463-1470 JJ denotes similar
T20091 1489-1494 NNS denotes acids
T20090 1457-1462 JJ denotes other
T20089 1453-1456 CC denotes and
T20088 1451-1453 , denotes ,
T20087 1444-1451 NN denotes alanine
T20086 1442-1444 , denotes ,
T20085 1433-1442 NN denotes glutamine
T20084 1431-1433 , denotes ,
T20083 1422-1431 NN denotes glutamate
T20082 1419-1421 IN denotes of
T20081 1412-1418 NNS denotes levels
T20080 1404-1411 VBN denotes reduced
T20079 1399-1403 IN denotes from
T20078 1388-1391 MD denotes may
T20077 1392-1398 VB denotes result
T20076 1368-1378 JJ denotes neurologic
T20075 1379-1387 NNS denotes symptoms
T20074 1362-1367 DT denotes These
T20073 1361-1574 sentence denotes These neurologic symptoms may result from reduced levels of glutamate, glutamine, alanine, and other similar neuroactive amino acids, which are considered the culprit for MSUD encephalopathies in patients [1,37].
T20072 1360-1361 . denotes .
T20071 1359-1360 -RRB- denotes ]
T20070 1358-1359 CD denotes 1
T20069 1357-1358 -LRB- denotes [
T20068 1344-1356 NNS denotes difficulties
T20067 1332-1343 JJ denotes respiratory
T20066 1328-1331 CC denotes and
T20065 1326-1328 , denotes ,
T20064 1318-1326 NN denotes activity
T20063 1312-1317 NN denotes motor
T20062 1309-1311 IN denotes of
T20061 1304-1308 NN denotes loss
T20060 1302-1304 , denotes ,
T20059 1294-1302 NN denotes lethargy
T20058 1292-1294 , denotes ,
T20057 1286-1292 NN denotes stupor
T20056 1284-1286 , denotes ,
T20055 1276-1284 NNS denotes seizures
T20054 1273-1275 IN denotes as
T20053 1268-1272 JJ denotes such
T20052 1256-1267 NN denotes dysfunction
T20051 1245-1255 JJ denotes neurologic
T20050 1242-1244 IN denotes of
T20049 1236-1241 NNS denotes signs
T20048 1228-1235 VBP denotes include
T20047 1222-1227 DT denotes These
T20046 1221-1361 sentence denotes These include signs of neurologic dysfunction such as seizures, stupor, lethargy, loss of motor activity, and respiratory difficulties [1].
T20045 1220-1221 . denotes .
T20044 1207-1211 NN denotes MSUD
T20043 1199-1206 JJ denotes classic
T20042 1212-1220 NNS denotes patients
T20041 1191-1198 JJ denotes newborn
T20040 1188-1190 IN denotes in
T20039 1183-1187 VBN denotes seen
T20038 1174-1182 NNS denotes symptoms
T20037 1153-1158 NN denotes mouse
T20036 1159-1163 NNS denotes pups
T20035 1142-1152 JJ denotes homozygous
T20034 1139-1141 IN denotes of
T20033 1164-1173 VBZ denotes resembles
T20032 1119-1129 JJ denotes phenotypic
T20031 1130-1138 NN denotes behavior
T20030 1115-1118 DT denotes The
T20029 1114-1221 sentence denotes The phenotypic behavior of homozygous mouse pups resembles symptoms seen in newborn classic MSUD patients.
T20028 1113-1114 . denotes .
T20027 1109-1113 NNS denotes pups
T20026 1098-1108 JJ denotes homozygous
T20025 1095-1097 IN denotes of
T20024 1083-1094 NNS denotes fibroblasts
T20023 1079-1082 CC denotes and
T20022 1073-1078 NN denotes liver
T20021 1070-1072 IN denotes in
T20020 1063-1069 JJ denotes absent
T20019 1059-1062 VBD denotes was
T20018 1048-1050 NN denotes E2
T20017 1051-1058 NN denotes protein
T20016 1033-1047 JJ denotes Immunoreactive
T20015 1032-1114 sentence denotes Immunoreactive E2 protein was absent in liver and fibroblasts of homozygous pups.
T20014 1031-1032 . denotes .
T20013 1027-1031 NN denotes BCAA
T20012 1013-1026 JJ denotes unmetabolized
T19940 636-641 NN denotes birth
T19939 633-635 IN denotes at
T19938 626-632 JJ denotes normal
T19937 617-625 VBD denotes appeared
T19936 613-616 CC denotes and
T19935 594-602 VBN denotes expected
T19934 603-612 NN denotes frequency
T19933 590-593 DT denotes the
T19932 587-589 IN denotes at
T19931 577-581 VBD denotes were
T19930 582-586 VBN denotes born
T19929 572-576 NNS denotes mice
T19928 563-571 NN denotes Knockout
T19927 562-642 sentence denotes Knockout mice were born at the expected frequency and appeared normal at birth.
T19926 561-562 . denotes .
T19925 557-561 NN denotes MSUD
T19924 549-556 JJ denotes classic
T19923 544-548 IN denotes with
T19922 537-543 NNS denotes humans
T19921 534-536 IN denotes to
T19920 526-533 JJ denotes similar
T19919 515-525 RB denotes strikingly
T19918 495-503 NN denotes knockout
T19917 504-511 NNS denotes animals
T19916 489-494 DT denotes these
T19915 486-488 IN denotes of
T19914 512-514 VBZ denotes is
T19913 476-485 NN denotes phenotype
T19912 472-475 DT denotes The
T19911 471-562 sentence denotes The phenotype of these knockout animals is strikingly similar to humans with classic MSUD.
T19910 470-471 . denotes .
T19909 465-470 NN denotes BCKDH
T19908 462-464 IN denotes of
T19907 451-453 NN denotes E2
T19906 454-461 NN denotes subunit
T19905 447-450 DT denotes the
T19904 444-446 IN denotes of
T19903 435-443 NN denotes knockout
T19902 430-434 NN denotes gene
T19901 427-429 IN denotes by
T19900 415-418 VBD denotes was
T19899 410-414 NN denotes MSUD
T19898 402-409 JJ denotes classic
T19897 399-401 IN denotes of
T19896 419-426 VBN denotes created
T19895 393-398 NN denotes model
T19894 389-392 PRP$ denotes Our
T19893 388-471 sentence denotes Our model of classic MSUD was created by gene knockout of the E2 subunit of BCKDH.
T19892 387-388 . denotes .
T19891 380-387 NN denotes disease
T19890 376-379 DT denotes the
T19889 373-375 IN denotes of
T19888 354-366 JJ denotes intermediate
T19887 350-353 CC denotes and
T19886 342-349 JJ denotes classic
T19885 367-372 NNS denotes forms
T19884 338-341 DT denotes the
T20232 2221-2345 sentence denotes To create a model of intermediate MSUD, we used a transgenic strategy to express human E2 in the liver of E2 knockout mice.
T20231 2220-2221 . denotes .
T20230 2219-2220 -RRB- denotes ]
T20229 2218-2219 CD denotes 1
T20228 2217-2218 -LRB- denotes [
T19883 333-337 IN denotes with
T19882 324-332 NNS denotes patients
T19881 319-323 NN denotes MSUD
T19880 316-318 IN denotes to
T19879 308-315 JJ denotes similar
T19878 303-307 RB denotes very
T19877 288-302 RB denotes phenotypically
T19876 284-287 VBP denotes are
T19875 279-283 WDT denotes that
T19874 266-271 NN denotes mouse
T19873 255-265 VBN denotes engineered
T19872 243-254 RB denotes genetically
T19871 272-278 NNS denotes models
T19870 239-242 CD denotes two
T19869 236-238 IN denotes of
T19868 219-235 NN denotes characterization
T19867 215-218 CC denotes and
T19866 203-214 NN denotes development
T19865 199-202 DT denotes the
T19864 187-189 PRP denotes we
T19863 185-187 , denotes ,
T19862 179-185 NN denotes report
T19861 174-178 DT denotes this
T19860 190-198 VBP denotes describe
T19859 171-173 IN denotes In
T19858 170-388 sentence denotes In this report, we describe the development and characterization of two genetically engineered mouse models that are phenotypically very similar to MSUD patients with the classic and intermediate forms of the disease.
T19857 169-170 . denotes .
T19856 156-160 VBN denotes been
T19855 152-155 RB denotes now
T19854 148-151 VBZ denotes has
T19853 161-169 VBN denotes overcome
T19852 141-147 NN denotes hurdle
T19851 136-140 DT denotes This
T19850 135-170 sentence denotes This hurdle has now been overcome.
T19849 134-135 . denotes .
T19848 127-134 NN denotes disease
T19847 123-126 DT denotes the
T19846 120-122 IN denotes of
T19845 107-113 NN denotes animal
T19844 98-106 JJ denotes accurate
T19843 96-98 , denotes ,
T19842 87-96 JJ denotes practical
T19841 114-119 NN denotes model
T19840 85-86 DT denotes a
T19839 82-84 IN denotes of
T19838 77-81 NN denotes lack
T19837 73-76 DT denotes the
T19836 64-67 VBZ denotes has
T19835 59-63 NN denotes MSUD
T19834 55-58 IN denotes for
T19833 44-54 NNS denotes treatments
T19832 40-43 JJ denotes new
T19831 29-39 VBG denotes developing
T19830 26-28 IN denotes in
T19829 68-72 VBN denotes been
T19828 13-18 JJ denotes major
T19827 19-25 NN denotes hurdle
T19826 11-12 DT denotes A
T20011 1010-1012 IN denotes of
T20010 997-1009 NN denotes accumulation
T20009 993-996 DT denotes the
T20008 989-992 IN denotes for
T20007 978-988 VBG denotes accounting
T20006 976-978 , denotes ,
T20005 964-976 JJ denotes undetectable
T20004 949-954 NN denotes mouse
T20003 955-959 NNS denotes pups
T20002 940-948 NN denotes knockout
T20001 929-939 JJ denotes homozygous
T20000 926-928 IN denotes of
T19999 919-925 NNS denotes livers
T19998 916-918 IN denotes in
T19997 910-915 NN denotes BCKDH
T19996 907-909 IN denotes of
T19995 960-963 VBD denotes was
T19994 898-906 NN denotes activity
T19993 894-897 DT denotes The
T19992 893-1032 sentence denotes The activity of BCKDH in livers of homozygous knockout mouse pups was undetectable, accounting for the accumulation of unmetabolized BCAA.
T19991 892-893 . denotes .
T19990 891-892 -RRB- denotes )
T19989 890-891 CD denotes 1
T19988 884-889 NN denotes Table
T19987 883-884 -LRB- denotes (
T19986 868-872 VBD denotes were
T19985 866-868 , denotes ,
T19984 862-866 NN denotes BCAA
T19983 859-861 IN denotes of
T19982 848-858 NN denotes metabolism
T19981 841-847 JJ denotes normal
T19980 836-840 IN denotes with
T19979 826-828 VBZ denotes is
T19978 829-835 VBN denotes linked
T19977 816-825 NN denotes synthesis
T19976 810-815 WP$ denotes whose
T19975 808-810 , denotes ,
T19974 799-808 NN denotes glutamine
T19973 795-798 CC denotes and
T19972 793-795 , denotes ,
T19971 784-793 NN denotes glutamate
T19970 782-784 , denotes ,
T19969 775-782 NN denotes alanine
T19968 763-768 NN denotes amino
T19967 769-774 NNS denotes acids
T19966 759-762 DT denotes the
T19965 756-758 IN denotes of
T19964 749-755 NNS denotes levels
T19963 747-749 , denotes ,
T19962 873-882 VBN denotes decreased
T19961 734-747 RB denotes Concomitantly
T19960 733-893 sentence denotes Concomitantly, levels of the amino acids alanine, glutamate, and glutamine, whose synthesis is linked with normal metabolism of BCAA, were decreased (Table 1).
T19959 732-733 . denotes .
T19958 724-732 JJ denotes elevated
T19957 715-723 RB denotes markedly
T19956 705-709 NN denotes BCAA
T19955 702-704 IN denotes of
T19954 695-701 NNS denotes levels
T19953 689-694 NN denotes blood
T19952 687-689 , denotes ,
T19951 679-687 NN denotes suckling
T19950 669-678 VBG denotes following
T19949 665-668 CC denotes and
T19948 659-664 NN denotes birth
T19947 656-658 IN denotes of
T19946 652-655 NN denotes day
T19945 650-651 DT denotes a
T19944 710-714 VBD denotes were
T19943 643-649 IN denotes Within
T19942 642-733 sentence denotes Within a day of birth and following suckling, blood levels of BCAA were markedly elevated.
T19941 641-642 . denotes .
T21164 7135-7136 . denotes .
T21163 7128-7135 NNS denotes enzymes
T21162 7115-7127 JJ denotes multisubunit
T21161 7112-7114 IN denotes in
T21160 7104-7111 NNS denotes defects
T21159 7101-7103 IN denotes to
T21158 7097-7100 IN denotes due
T21157 7073-7086 JJ denotes mitochondrial
T21156 7087-7096 NNS denotes disorders
T21155 7067-7072 JJ denotes other
T21154 7064-7066 IN denotes to
T21153 7051-7063 JJ denotes transferable
T21152 7047-7050 CC denotes and
T21151 7036-7046 JJ denotes applicable
T21150 7028-7032 RB denotes also
T21149 7023-7027 MD denotes will
T21148 7018-7022 RB denotes here
T21147 7008-7017 VBN denotes described
T21146 6996-7000 NN denotes MSUD
T21145 7001-7007 NNS denotes models
T21144 6992-6995 DT denotes the
T21143 6987-6991 IN denotes with
T21142 6979-6986 NNS denotes studies
T21141 6974-6978 IN denotes from
T21140 6967-6973 VBN denotes gained
T21139 6957-6966 NN denotes knowledge
T21138 6953-6956 CC denotes and
T21137 6941-6952 NN denotes information
T21136 6937-6940 DT denotes the
T21135 6935-6937 , denotes ,
T21134 7033-7035 VB denotes be
T21133 6929-6935 RB denotes Lastly
T21132 6928-7136 sentence denotes Lastly, the information and knowledge gained from studies with the MSUD models described here will also be applicable and transferable to other mitochondrial disorders due to defects in multisubunit enzymes.
T21131 6927-6928 . denotes .
T21130 6920-6927 NN denotes biology
T21129 6909-6919 JJ denotes structural
T21128 6906-6908 IN denotes of
T21127 6898-6905 NNS denotes studies
T21126 6894-6897 IN denotes for
T21125 6876-6884 JJ denotes valuable
T21124 6885-6893 NN denotes resource
T21123 6874-6875 DT denotes a
T21122 6862-6865 MD denotes may
R4968 T19826 T19827 det A,hurdle
R4969 T19827 T19829 nsubj hurdle,been
R4970 T19828 T19827 amod major,hurdle
R4971 T19830 T19827 prep in,hurdle
R4972 T19831 T19830 pcomp developing,in
R4973 T19832 T19833 amod new,treatments
R4974 T19833 T19831 dobj treatments,developing
R4975 T19834 T19833 prep for,treatments
R4976 T19835 T19834 pobj MSUD,for
R4977 T19836 T19829 aux has,been
R4978 T19837 T19838 det the,lack
R4979 T19838 T19829 attr lack,been
R4980 T19839 T19838 prep of,lack
R4981 T19840 T19841 det a,model
R4982 T19841 T19839 pobj model,of
R4983 T19842 T19841 amod practical,model
R4984 T19843 T19841 punct ", ",model
R4985 T19844 T19841 amod accurate,model
R4986 T19845 T19841 compound animal,model
R4987 T19846 T19841 prep of,model
R4988 T19847 T19848 det the,disease
R4989 T19848 T19846 pobj disease,of
R4990 T19849 T19829 punct .,been
R4991 T19851 T19852 det This,hurdle
R4992 T19852 T19853 nsubjpass hurdle,overcome
R4993 T19854 T19853 aux has,overcome
R4994 T19855 T19853 advmod now,overcome
R4995 T19856 T19853 auxpass been,overcome
R4996 T19857 T19853 punct .,overcome
R4997 T19859 T19860 prep In,describe
R4998 T19861 T19862 det this,report
R4999 T19862 T19859 pobj report,In
R5000 T19863 T19860 punct ", ",describe
R5001 T19864 T19860 nsubj we,describe
R5002 T19865 T19866 det the,development
R5003 T19866 T19860 dobj development,describe
R5004 T19867 T19866 cc and,development
R5005 T19868 T19866 conj characterization,development
R5006 T19869 T19866 prep of,development
R5007 T19870 T19871 nummod two,models
R5008 T19871 T19869 pobj models,of
R5009 T19872 T19873 advmod genetically,engineered
R5010 T19873 T19871 amod engineered,models
R5011 T19874 T19871 compound mouse,models
R5012 T19875 T19876 dep that,are
R5013 T19876 T19871 relcl are,models
R5014 T19877 T19876 advmod phenotypically,are
R5015 T19878 T19879 advmod very,similar
R5016 T19879 T19876 acomp similar,are
R5017 T19880 T19879 prep to,similar
R5018 T19881 T19882 compound MSUD,patients
R5019 T19882 T19880 pobj patients,to
R5020 T19883 T19882 prep with,patients
R5021 T19884 T19885 det the,forms
R5022 T19885 T19883 pobj forms,with
R5023 T19886 T19885 amod classic,forms
R5024 T19887 T19886 cc and,classic
R5025 T19888 T19886 conj intermediate,classic
R5026 T19889 T19885 prep of,forms
R5027 T19890 T19891 det the,disease
R5028 T19891 T19889 pobj disease,of
R5029 T19892 T19860 punct .,describe
R5030 T19894 T19895 poss Our,model
R5031 T19895 T19896 nsubjpass model,created
R5032 T19897 T19895 prep of,model
R5033 T19898 T19899 amod classic,MSUD
R5034 T19899 T19897 pobj MSUD,of
R5035 T19900 T19896 auxpass was,created
R5036 T19901 T19896 prep by,created
R5037 T19902 T19903 compound gene,knockout
R5038 T19903 T19901 pobj knockout,by
R5039 T19904 T19903 prep of,knockout
R5040 T19905 T19906 det the,subunit
R5041 T19906 T19904 pobj subunit,of
R5042 T19907 T19906 compound E2,subunit
R5043 T19908 T19906 prep of,subunit
R5044 T19909 T19908 pobj BCKDH,of
R5045 T19910 T19896 punct .,created
R5046 T19912 T19913 det The,phenotype
R5047 T19913 T19914 nsubj phenotype,is
R5048 T19915 T19913 prep of,phenotype
R5049 T19916 T19917 det these,animals
R5050 T19917 T19915 pobj animals,of
R5051 T19918 T19917 compound knockout,animals
R5052 T19919 T19920 advmod strikingly,similar
R5053 T19920 T19914 acomp similar,is
R5054 T19921 T19920 prep to,similar
R5055 T19922 T19921 pobj humans,to
R5056 T19923 T19922 prep with,humans
R5057 T19924 T19925 amod classic,MSUD
R5058 T19925 T19923 pobj MSUD,with
R5059 T19926 T19914 punct .,is
R5060 T19928 T19929 compound Knockout,mice
R5061 T19929 T19930 nsubjpass mice,born
R5062 T19931 T19930 auxpass were,born
R5063 T19932 T19930 prep at,born
R5064 T19933 T19934 det the,frequency
R5065 T19934 T19932 pobj frequency,at
R5066 T19935 T19934 amod expected,frequency
R5067 T19936 T19930 cc and,born
R5068 T19937 T19930 conj appeared,born
R5069 T19938 T19937 oprd normal,appeared
R5070 T19939 T19937 prep at,appeared
R5071 T19940 T19939 pobj birth,at
R5072 T19941 T19930 punct .,born
R5073 T19943 T19944 prep Within,were
R5074 T19945 T19946 det a,day
R5075 T19946 T19943 pobj day,Within
R5076 T19947 T19946 prep of,day
R5077 T19948 T19947 pobj birth,of
R5078 T19949 T19943 cc and,Within
R5079 T19950 T19943 conj following,Within
R5080 T19951 T19950 pobj suckling,following
R5081 T19952 T19944 punct ", ",were
R5082 T19953 T19954 compound blood,levels
R5083 T19954 T19944 nsubj levels,were
R5084 T19955 T19954 prep of,levels
R5085 T19956 T19955 pobj BCAA,of
R5086 T19957 T19958 advmod markedly,elevated
R5087 T19958 T19944 acomp elevated,were
R5088 T19959 T19944 punct .,were
R5089 T19961 T19962 advmod Concomitantly,decreased
R5090 T19963 T19962 punct ", ",decreased
R5091 T19964 T19962 nsubjpass levels,decreased
R5092 T19965 T19964 prep of,levels
R5093 T19966 T19967 det the,acids
R5094 T19967 T19965 pobj acids,of
R5095 T19968 T19967 compound amino,acids
R5096 T19969 T19967 appos alanine,acids
R5097 T19970 T19969 punct ", ",alanine
R5098 T19971 T19969 conj glutamate,alanine
R5099 T19972 T19971 punct ", ",glutamate
R5100 T19973 T19971 cc and,glutamate
R5101 T19974 T19971 conj glutamine,glutamate
R5102 T19975 T19967 punct ", ",acids
R5103 T19976 T19977 poss whose,synthesis
R5104 T19977 T19978 dep synthesis,linked
R5105 T19978 T19967 relcl linked,acids
R5106 T19979 T19978 auxpass is,linked
R5107 T19980 T19978 prep with,linked
R5108 T19981 T19982 amod normal,metabolism
R5109 T19982 T19980 pobj metabolism,with
R5110 T19983 T19982 prep of,metabolism
R5111 T19984 T19983 pobj BCAA,of
R5112 T19985 T19962 punct ", ",decreased
R5113 T19986 T19962 auxpass were,decreased
R5114 T19987 T19988 punct (,Table
R5115 T19988 T19962 parataxis Table,decreased
R5116 T19989 T19988 nummod 1,Table
R5117 T19990 T19988 punct ),Table
R5118 T19991 T19962 punct .,decreased
R5119 T19993 T19994 det The,activity
R5120 T19994 T19995 nsubj activity,was
R5121 T19996 T19994 prep of,activity
R5122 T19997 T19996 pobj BCKDH,of
R5123 T19998 T19994 prep in,activity
R5124 T19999 T19998 pobj livers,in
R5125 T20000 T19999 prep of,livers
R5126 T20001 T20002 amod homozygous,knockout
R5127 T20002 T20003 compound knockout,pups
R5128 T20003 T20000 pobj pups,of
R5129 T20004 T20003 compound mouse,pups
R5130 T20005 T19995 acomp undetectable,was
R5131 T20006 T19995 punct ", ",was
R5132 T20007 T19995 advcl accounting,was
R5133 T20008 T20007 prep for,accounting
R5134 T20009 T20010 det the,accumulation
R5135 T20010 T20008 pobj accumulation,for
R5136 T20011 T20010 prep of,accumulation
R5137 T20012 T20013 amod unmetabolized,BCAA
R5138 T20013 T20011 pobj BCAA,of
R5139 T20014 T19995 punct .,was
R5140 T20016 T20017 amod Immunoreactive,protein
R5141 T20017 T20019 nsubj protein,was
R5142 T20018 T20017 compound E2,protein
R5143 T20020 T20019 acomp absent,was
R5144 T20021 T20019 prep in,was
R5145 T20022 T20021 pobj liver,in
R5146 T20023 T20022 cc and,liver
R5147 T20024 T20022 conj fibroblasts,liver
R5148 T20025 T20022 prep of,liver
R5149 T20026 T20027 amod homozygous,pups
R5150 T20027 T20025 pobj pups,of
R5151 T20028 T20019 punct .,was
R5152 T20030 T20031 det The,behavior
R5153 T20031 T20033 nsubj behavior,resembles
R5154 T20032 T20031 amod phenotypic,behavior
R5155 T20034 T20031 prep of,behavior
R5156 T20035 T20036 amod homozygous,pups
R5157 T20036 T20034 pobj pups,of
R5158 T20037 T20036 compound mouse,pups
R5159 T20038 T20033 dobj symptoms,resembles
R5160 T20039 T20038 acl seen,symptoms
R5161 T20040 T20039 prep in,seen
R5162 T20041 T20042 amod newborn,patients
R5163 T20042 T20040 pobj patients,in
R5164 T20043 T20042 amod classic,patients
R5165 T20044 T20042 compound MSUD,patients
R5166 T20045 T20033 punct .,resembles
R5167 T20047 T20048 nsubj These,include
R5168 T20049 T20048 dobj signs,include
R5169 T20050 T20049 prep of,signs
R5170 T20051 T20052 amod neurologic,dysfunction
R5171 T20052 T20050 pobj dysfunction,of
R5172 T20053 T20054 amod such,as
R5173 T20054 T20049 prep as,signs
R5174 T20055 T20054 pobj seizures,as
R5175 T20056 T20055 punct ", ",seizures
R5176 T20057 T20055 conj stupor,seizures
R5177 T20058 T20057 punct ", ",stupor
R5178 T20059 T20057 conj lethargy,stupor
R5179 T20060 T20059 punct ", ",lethargy
R5180 T20061 T20059 conj loss,lethargy
R5181 T20062 T20061 prep of,loss
R5182 T20063 T20064 compound motor,activity
R5183 T20064 T20062 pobj activity,of
R5184 T20065 T20061 punct ", ",loss
R5185 T20066 T20061 cc and,loss
R5186 T20067 T20068 amod respiratory,difficulties
R5187 T20068 T20061 conj difficulties,loss
R5188 T20069 T20070 punct [,1
R5189 T20070 T20048 parataxis 1,include
R5190 T20071 T20070 punct ],1
R5191 T20072 T20048 punct .,include
R5192 T20074 T20075 det These,symptoms
R5193 T20075 T20077 nsubj symptoms,result
R5194 T20076 T20075 amod neurologic,symptoms
R5195 T20078 T20077 aux may,result
R5196 T20079 T20077 prep from,result
R5197 T20080 T20081 amod reduced,levels
R5198 T20081 T20079 pobj levels,from
R5199 T20082 T20081 prep of,levels
R5200 T20083 T20082 pobj glutamate,of
R5201 T20084 T20083 punct ", ",glutamate
R5202 T20085 T20083 conj glutamine,glutamate
R5203 T20086 T20085 punct ", ",glutamine
R5204 T20087 T20085 conj alanine,glutamine
R5205 T20088 T20087 punct ", ",alanine
R5206 T20089 T20087 cc and,alanine
R5207 T20090 T20091 amod other,acids
R5208 T20091 T20087 conj acids,alanine
R5209 T20092 T20091 amod similar,acids
R5210 T20093 T20091 amod neuroactive,acids
R5211 T20094 T20091 compound amino,acids
R5212 T20095 T20083 punct ", ",glutamate
R5213 T20096 T20097 dep which,considered
R5214 T20097 T20083 relcl considered,glutamate
R5215 T20098 T20097 auxpass are,considered
R5216 T20099 T20100 det the,culprit
R5217 T20100 T20097 oprd culprit,considered
R5218 T20101 T20100 prep for,culprit
R5219 T20102 T20103 compound MSUD,encephalopathies
R5220 T20103 T20101 pobj encephalopathies,for
R5221 T20104 T20100 prep in,culprit
R5222 T20105 T20104 pobj patients,in
R5223 T20106 T20107 punct [,37
R5224 T20107 T20077 parataxis 37,result
R5225 T20108 T20107 nummod 1,37
R5226 T20109 T20107 punct ",",37
R5227 T20110 T20107 punct ],37
R5228 T20111 T20077 punct .,result
R5229 T20113 T20114 advmod Finally,died
R5230 T20115 T20114 punct ", ",died
R5231 T20116 T20117 advmod nearly,all
R5232 T20117 T20114 nsubj all,died
R5233 T20118 T20117 prep of,all
R5234 T20119 T20120 det the,pups
R5235 T20120 T20118 pobj pups,of
R5236 T20121 T20120 amod homozygous,pups
R5237 T20122 T20114 prep within,died
R5238 T20123 T20124 nummod 72,hours
R5239 T20124 T20122 pobj hours,within
R5240 T20125 T20124 prep of,hours
R5241 T20126 T20125 pobj birth,of
R5242 T20127 T20114 punct .,died
R5243 T20129 T20130 det This,lethality
R5244 T20130 T20132 nsubj lethality,is
R5245 T20131 T20130 amod neonatal,lethality
R5246 T20133 T20134 advmod likely,due
R5247 T20134 T20132 prep due,is
R5248 T20135 T20134 pcomp to,due
R5249 T20136 T20137 det the,accumulation
R5250 T20137 T20134 pobj accumulation,due
R5251 T20138 T20137 prep of,accumulation
R5252 T20139 T20138 pobj BCAA,of
R5253 T20140 T20137 prep to,accumulation
R5254 T20141 T20142 amod neurotoxic,levels
R5255 T20142 T20140 pobj levels,to
R5256 T20143 T20142 punct ", ",levels
R5257 T20144 T20142 conj ketoacidosis,levels
R5258 T20145 T20144 punct ", ",ketoacidosis
R5259 T20146 T20147 compound brain,edema
R5260 T20147 T20144 conj edema,ketoacidosis
R5261 T20148 T20147 punct ", ",edema
R5262 T20149 T20147 conj dehydration,edema
R5263 T20150 T20149 punct ", ",dehydration
R5264 T20151 T20149 cc and,dehydration
R5265 T20152 T20149 conj malnutrition,dehydration
R5266 T20153 T20154 mark as,observed
R5267 T20154 T20132 advcl observed,is
R5268 T20155 T20154 prep in,observed
R5269 T20156 T20157 det the,calf
R5270 T20157 T20155 pobj calf,in
R5271 T20158 T20157 compound MSUD,calf
R5272 T20159 T20160 punct [,17
R5273 T20160 T20157 parataxis 17,calf
R5274 T20161 T20160 punct ],17
R5275 T20162 T20155 cc and,in
R5276 T20163 T20155 conj in,in
R5277 T20164 T20165 amod classic,patients
R5278 T20165 T20163 pobj patients,in
R5279 T20166 T20165 compound MSUD,patients
R5280 T20167 T20168 punct [,1
R5281 T20168 T20165 parataxis 1,patients
R5282 T20169 T20168 punct ],1
R5283 T20170 T20132 punct .,is
R5284 T20172 T20173 amod Heterozygous,knockouts
R5285 T20173 T20174 nsubj knockouts,were
R5286 T20175 T20174 acomp normal,were
R5287 T20176 T20174 cc and,were
R5288 T20177 T20174 conj had,were
R5289 T20178 T20179 amod normal,levels
R5290 T20179 T20177 dobj levels,had
R5291 T20180 T20179 prep of,levels
R5292 T20181 T20180 pobj BCAA,of
R5293 T20182 T20177 prep despite,had
R5294 T20183 T20182 pcomp having,despite
R5295 T20184 T20185 advmod approximately,half
R5296 T20185 T20183 dobj half,having
R5297 T20186 T20185 prep of,half
R5298 T20187 T20188 nmod BCKDH,activity
R5299 T20188 T20186 pobj activity,of
R5300 T20189 T20188 amod enzymatic,activity
R5301 T20190 T20174 punct .,were
R5302 T20192 T20193 prep From,represents
R5303 T20194 T20195 det the,studies
R5304 T20195 T20192 pobj studies,From
R5305 T20196 T20195 compound characterization,studies
R5306 T20197 T20195 acl completed,studies
R5307 T20198 T20199 advmod thus,far
R5308 T20199 T20197 advmod far,completed
R5309 T20200 T20193 punct ", ",represents
R5310 T20201 T20202 det the,mouse
R5311 T20202 T20193 nsubj mouse,represents
R5312 T20203 T20204 amod null,mutation
R5313 T20204 T20202 compound mutation,mouse
R5314 T20205 T20193 advmod accurately,represents
R5315 T20206 T20207 det a,model
R5316 T20207 T20193 dobj model,represents
R5317 T20208 T20207 prep of,model
R5318 T20209 T20210 amod classic,MSUD
R5319 T20210 T20208 pobj MSUD,of
R5320 T20211 T20193 cc and,represents
R5321 T20212 T20193 conj appears,represents
R5322 T20213 T20214 aux to,be
R5323 T20214 T20212 xcomp be,appears
R5324 T20215 T20216 det a,model
R5325 T20216 T20214 attr model,be
R5326 T20217 T20216 amod faithful,model
R5327 T20218 T20216 prep of,model
R5328 T20219 T20220 det the,disease
R5329 T20220 T20218 pobj disease,of
R5330 T20221 T20220 amod human,disease
R5331 T20222 T20214 prep with,be
R5332 T20223 T20222 pobj respect,with
R5333 T20224 T20223 prep to,respect
R5334 T20225 T20226 amod several,phenotypes
R5335 T20226 T20224 pobj phenotypes,to
R5336 T20227 T20226 amod biochemical,phenotypes
R5337 T20228 T20229 punct [,1
R5338 T20229 T20212 parataxis 1,appears
R5339 T20230 T20229 punct ],1
R5340 T20231 T20193 punct .,represents
R5341 T20233 T20234 aux To,create
R5342 T20234 T20235 advcl create,used
R5343 T20236 T20237 det a,model
R5344 T20237 T20234 dobj model,create
R5345 T20238 T20237 prep of,model
R5346 T20239 T20240 amod intermediate,MSUD
R5347 T20240 T20238 pobj MSUD,of
R5348 T20241 T20235 punct ", ",used
R5349 T20242 T20235 nsubj we,used
R5350 T20243 T20244 det a,strategy
R5351 T20244 T20235 dobj strategy,used
R5352 T20245 T20244 amod transgenic,strategy
R5353 T20246 T20247 aux to,express
R5354 T20247 T20235 advcl express,used
R5355 T20248 T20249 amod human,E2
R5356 T20249 T20247 dobj E2,express
R5357 T20250 T20247 prep in,express
R5358 T20251 T20252 det the,liver
R5359 T20252 T20250 pobj liver,in
R5360 T20253 T20252 prep of,liver
R5361 T20254 T20255 compound E2,mice
R5362 T20255 T20253 pobj mice,of
R5363 T20256 T20255 compound knockout,mice
R5364 T20257 T20235 punct .,used
R5365 T20259 T20260 mark As,mentioned
R5366 T20260 T20261 advcl mentioned,die
R5367 T20262 T20260 advmod above,mentioned
R5368 T20263 T20261 punct ", ",die
R5369 T20264 T20265 compound E2,knockouts
R5370 T20265 T20261 nsubj knockouts,die
R5371 T20266 T20265 prep without,knockouts
R5372 T20267 T20268 npadvmod transgene,derived
R5373 T20268 T20269 amod derived,E2
R5374 T20269 T20266 pobj E2,without
R5375 T20270 T20261 prep during,die
R5376 T20271 T20272 det the,period
R5377 T20272 T20270 pobj period,during
R5378 T20273 T20274 amod early,neonatal
R5379 T20274 T20272 amod neonatal,period
R5380 T20275 T20261 punct .,die
R5381 T20277 T20278 nsubj We,show
R5382 T20279 T20280 mark that,is
R5383 T20280 T20278 ccomp is,show
R5384 T20281 T20280 nsubj expression,is
R5385 T20282 T20281 prep of,expression
R5386 T20283 T20284 det a,transgene
R5387 T20284 T20282 pobj transgene,of
R5388 T20285 T20284 amod human,transgene
R5389 T20286 T20284 compound E2,transgene
R5390 T20287 T20281 prep in,expression
R5391 T20288 T20289 det the,liver
R5392 T20289 T20287 pobj liver,in
R5393 T20290 T20289 prep of,liver
R5394 T20291 T20292 det these,mice
R5395 T20292 T20290 pobj mice,of
R5396 T20293 T20292 compound knockout,mice
R5397 T20294 T20280 acomp able,is
R5398 T20295 T20296 aux to,rescue
R5399 T20296 T20294 xcomp rescue,able
R5400 T20297 T20298 det the,lethality
R5401 T20298 T20296 dobj lethality,rescue
R5402 T20299 T20298 amod neonatal,lethality
R5403 T20300 T20278 punct .,show
R5404 T20302 T20303 nsubj Many,survived
R5405 T20304 T20302 prep of,Many
R5406 T20305 T20306 det the,mice
R5407 T20306 T20304 pobj mice,of
R5408 T20307 T20306 compound rescue,mice
R5409 T20308 T20303 prep to,survived
R5410 T20309 T20308 pobj adulthood,to
R5411 T20310 T20303 punct .,survived
R5412 T20312 T20313 nsubj It,is
R5413 T20314 T20313 acomp interesting,is
R5414 T20315 T20316 aux to,note
R5415 T20316 T20313 xcomp note,is
R5416 T20317 T20318 mark that,was
R5417 T20318 T20316 ccomp was,note
R5418 T20319 T20318 prep in,was
R5419 T20320 T20321 det these,lines
R5420 T20321 T20319 pobj lines,in
R5421 T20322 T20321 compound mouse,lines
R5422 T20323 T20324 advmod only,6
R5423 T20324 T20328 nummod 6,%
R5424 T20325 T20324 punct ~,6
R5425 T20326 T20324 quantmod 5,6
R5426 T20327 T20324 punct –,6
R5427 T20328 T20318 nsubj %,was
R5428 T20329 T20328 prep of,%
R5429 T20330 T20331 amod normal,activity
R5430 T20331 T20329 pobj activity,of
R5431 T20332 T20331 compound BCKDH,activity
R5432 T20333 T20331 prep in,activity
R5433 T20334 T20335 det the,liver
R5434 T20335 T20333 pobj liver,in
R5435 T20336 T20318 acomp sufficient,was
R5436 T20337 T20338 aux to,allow
R5437 T20338 T20336 xcomp allow,sufficient
R5438 T20339 T20338 dobj survival,allow
R5439 T20340 T20313 punct .,is
R5440 T20342 T20343 nsubj We,demonstrated
R5441 T20344 T20343 advmod also,demonstrated
R5442 T20345 T20346 mark that,were
R5443 T20346 T20343 ccomp were,demonstrated
R5444 T20347 T20348 compound BCAA,levels
R5445 T20348 T20346 nsubj levels,were
R5446 T20349 T20348 prep in,levels
R5447 T20350 T20349 pobj blood,in
R5448 T20351 T20350 prep of,blood
R5449 T20352 T20353 det these,mice
R5450 T20353 T20351 pobj mice,of
R5451 T20354 T20353 compound rescue,mice
R5452 T20355 T20346 acomp intermediate,were
R5453 T20356 T20346 prep between,were
R5454 T20357 T20356 pobj controls,between
R5455 T20358 T20357 cc and,controls
R5456 T20359 T20357 conj knockouts,controls
R5457 T20360 T20343 punct .,demonstrated
R5458 T20362 T20363 det The,hallmarks
R5459 T20363 T20364 nsubj hallmarks,are
R5460 T20365 T20363 prep of,hallmarks
R5461 T20366 T20367 amod intermediate,patients
R5462 T20367 T20365 pobj patients,of
R5463 T20368 T20367 nmod MSUD,patients
R5464 T20369 T20367 amod human,patients
R5465 T20370 T20371 advmod persistently,increased
R5466 T20371 T20372 amod increased,levels
R5467 T20372 T20364 attr levels,are
R5468 T20373 T20372 prep of,levels
R5469 T20374 T20375 nmod BCAA,activity
R5470 T20375 T20373 pobj activity,of
R5471 T20376 T20374 cc and,BCAA
R5472 T20377 T20374 conj BCKDH,BCAA
R5473 T20378 T20372 prep in,levels
R5474 T20379 T20380 det the,range
R5475 T20380 T20378 pobj range,in
R5476 T20381 T20380 prep of,range
R5477 T20382 T20383 quantmod 3,30
R5478 T20383 T20385 nummod 30,%
R5479 T20384 T20383 punct –,30
R5480 T20385 T20381 pobj %,of
R5481 T20386 T20385 prep of,%
R5482 T20387 T20386 pobj normal,of
R5483 T20388 T20389 punct [,1
R5484 T20389 T20364 parataxis 1,are
R5485 T20390 T20389 punct ],1
R5486 T20391 T20364 punct .,are
R5487 T20393 T20394 prep Because,represent
R5488 T20395 T20393 pcomp of,Because
R5489 T20396 T20397 det the,similarities
R5490 T20397 T20393 pobj similarities,Because
R5491 T20398 T20397 amod phenotypic,similarities
R5492 T20399 T20397 prep of,similarities
R5493 T20400 T20401 det the,mice
R5494 T20401 T20399 pobj mice,of
R5495 T20402 T20401 compound rescue,mice
R5496 T20403 T20397 prep to,similarities
R5497 T20404 T20405 det the,patients
R5498 T20405 T20403 pobj patients,to
R5499 T20406 T20405 amod human,patients
R5500 T20407 T20394 punct ", ",represent
R5501 T20408 T20409 det these,mice
R5502 T20409 T20394 nsubj mice,represent
R5503 T20410 T20411 advmod genetically,engineered
R5504 T20411 T20409 amod engineered,mice
R5505 T20412 T20413 det a,model
R5506 T20413 T20394 dobj model,represent
R5507 T20414 T20413 amod useful,model
R5508 T20415 T20416 amod small,animal
R5509 T20416 T20413 compound animal,model
R5510 T20417 T20413 prep of,model
R5511 T20418 T20419 det the,form
R5512 T20419 T20417 pobj form,of
R5513 T20420 T20419 amod intermediate,form
R5514 T20421 T20419 prep of,form
R5515 T20422 T20421 pobj MSUD,of
R5516 T20423 T20394 punct .,represent
R5517 T20425 T20426 det The,approach
R5518 T20426 T20428 nsubjpass approach,based
R5519 T20427 T20426 amod transgenic,approach
R5520 T20429 T20430 dep that,used
R5521 T20430 T20426 relcl used,approach
R5522 T20431 T20430 auxpass was,used
R5523 T20432 T20433 aux to,create
R5524 T20433 T20430 advcl create,used
R5525 T20434 T20435 det the,model
R5526 T20435 T20433 dobj model,create
R5527 T20436 T20435 amod intermediate,model
R5528 T20437 T20435 compound MSUD,model
R5529 T20438 T20428 auxpass was,based
R5530 T20439 T20428 prep on,based
R5531 T20440 T20441 det the,system
R5532 T20441 T20439 pobj system,on
R5533 T20442 T20443 npadvmod tetracycline,regulated
R5534 T20443 T20441 amod regulated,system
R5535 T20444 T20445 compound gene,switch
R5536 T20445 T20441 compound switch,system
R5537 T20446 T20447 dep that,used
R5538 T20447 T20441 relcl used,system
R5539 T20448 T20447 aux has,used
R5540 T20449 T20447 auxpass been,used
R5541 T20450 T20447 prep with,used
R5542 T20451 T20452 amod great,success
R5543 T20452 T20450 pobj success,with
R5544 T20453 T20447 prep in,used
R5545 T20454 T20455 amod other,studies
R5546 T20455 T20453 pobj studies,in
R5547 T20456 T20447 punct ", ",used
R5548 T20457 T20447 prep for,used
R5549 T20458 T20457 pobj example,for
R5550 T20459 T20460 punct [,34
R5551 T20460 T20428 parataxis 34,based
R5552 T20461 T20460 punct ],34
R5553 T20462 T20428 punct .,based
R5554 T20464 T20465 det The,strategy
R5555 T20465 T20466 nsubj strategy,was
R5556 T20467 T20465 prep behind,strategy
R5557 T20468 T20469 poss our,approach
R5558 T20469 T20467 pobj approach,behind
R5559 T20470 T20471 aux to,create
R5560 T20471 T20466 xcomp create,was
R5561 T20472 T20471 dobj mice,create
R5562 T20473 T20472 prep with,mice
R5563 T20474 T20475 det an,phenotype
R5564 T20475 T20473 pobj phenotype,with
R5565 T20476 T20475 amod intermediate,phenotype
R5566 T20477 T20475 compound MSUD,phenotype
R5567 T20478 T20479 dep that,converted
R5568 T20479 T20475 relcl converted,phenotype
R5569 T20480 T20479 aux could,converted
R5570 T20481 T20479 auxpass be,converted
R5571 T20482 T20479 prep to,converted
R5572 T20483 T20484 det the,phenotype
R5573 T20484 T20482 pobj phenotype,to
R5574 T20485 T20484 amod classic,phenotype
R5575 T20486 T20479 prep at,converted
R5576 T20487 T20488 det the,investigator
R5577 T20488 T20489 poss investigator,discretion
R5578 T20489 T20486 pobj discretion,at
R5579 T20490 T20488 case 's,investigator
R5580 T20491 T20479 prep by,converted
R5581 T20492 T20491 pcomp turning,by
R5582 T20493 T20492 prt off,turning
R5583 T20494 T20495 det the,transgene
R5584 T20495 T20492 dobj transgene,turning
R5585 T20496 T20495 amod rescuing,transgene
R5586 T20497 T20466 punct .,was
R5587 T20499 T20500 advmod However,were
R5588 T20501 T20500 punct ", ",were
R5589 T20502 T20500 nsubj we,were
R5590 T20503 T20500 acomp unable,were
R5591 T20504 T20505 aux to,turn
R5592 T20505 T20503 xcomp turn,unable
R5593 T20506 T20505 advmod consistently,turn
R5594 T20507 T20505 prt off,turn
R5595 T20508 T20509 det the,cassette
R5596 T20509 T20505 dobj cassette,turn
R5597 T20510 T20509 amod human,cassette
R5598 T20511 T20509 compound E2,cassette
R5599 T20512 T20509 compound expression,cassette
R5600 T20513 T20505 prep by,turn
R5601 T20514 T20515 det the,analogue
R5602 T20515 T20513 pobj analogue,by
R5603 T20516 T20515 amod potent,analogue
R5604 T20517 T20515 compound tetracycline,analogue
R5605 T20518 T20515 punct ", ",analogue
R5606 T20519 T20515 appos doxycycline,analogue
R5607 T20520 T20505 prep in,turn
R5608 T20521 T20520 pobj either,in
R5609 T20522 T20521 prep of,either
R5610 T20523 T20524 det the,lines
R5611 T20524 T20522 pobj lines,of
R5612 T20525 T20524 nummod two,lines
R5613 T20526 T20524 amod transgenic,lines
R5614 T20527 T20524 acl tested,lines
R5615 T20528 T20529 punct (,shown
R5616 T20529 T20500 parataxis shown,were
R5617 T20530 T20529 nsubj data,shown
R5618 T20531 T20529 neg not,shown
R5619 T20532 T20529 punct ),shown
R5620 T20533 T20500 punct .,were
R5621 T20535 T20536 det The,reason
R5622 T20536 T20537 nsubj reason,is
R5623 T20538 T20539 det these,lines
R5624 T20539 T20541 nsubj lines,were
R5625 T20540 T20539 nummod two,lines
R5626 T20541 T20536 advcl were,reason
R5627 T20542 T20541 acomp unresponsive,were
R5628 T20543 T20542 prep to,unresponsive
R5629 T20544 T20543 pobj doxycycline,to
R5630 T20545 T20537 acomp unknown,is
R5631 T20546 T20537 punct .,is
R5632 T20548 T20549 nsubj It,is
R5633 T20550 T20549 acomp conceivable,is
R5634 T20551 T20552 mark that,was
R5635 T20552 T20549 ccomp was,is
R5636 T20553 T20554 det the,site
R5637 T20554 T20552 nsubj site,was
R5638 T20555 T20554 compound integration,site
R5639 T20556 T20554 prep of,site
R5640 T20557 T20558 det the,transgenes
R5641 T20558 T20556 pobj transgenes,of
R5642 T20559 T20552 neg not,was
R5643 T20560 T20552 acomp permissive,was
R5644 T20561 T20560 prep for,permissive
R5645 T20562 T20563 amod regulated,expression
R5646 T20563 T20561 pobj expression,for
R5647 T20564 T20549 punct .,is
R5648 T20566 T20567 nsubjpass Screening,needed
R5649 T20568 T20566 prep of,Screening
R5650 T20569 T20570 amod additional,lines
R5651 T20570 T20568 pobj lines,of
R5652 T20571 T20567 aux may,needed
R5653 T20572 T20567 auxpass be,needed
R5654 T20573 T20574 aux to,find
R5655 T20574 T20567 advcl find,needed
R5656 T20575 T20574 dobj lines,find
R5657 T20576 T20577 dep that,allow
R5658 T20577 T20575 relcl allow,lines
R5659 T20578 T20577 prep for,allow
R5660 T20579 T20578 pobj survival,for
R5661 T20580 T20577 cc and,allow
R5662 T20581 T20582 aux can,regulated
R5663 T20582 T20577 conj regulated,allow
R5664 T20583 T20582 advmod also,regulated
R5665 T20584 T20582 auxpass be,regulated
R5666 T20585 T20567 punct .,needed
R5667 T20587 T20588 det The,models
R5668 T20588 T20589 nsubj models,are
R5669 T20590 T20588 acl described,models
R5670 T20591 T20590 prep in,described
R5671 T20592 T20593 det this,communication
R5672 T20593 T20591 pobj communication,in
R5673 T20594 T20595 amod important,advances
R5674 T20595 T20589 attr advances,are
R5675 T20596 T20595 prep over,advances
R5676 T20597 T20598 det the,models
R5677 T20598 T20596 pobj models,over
R5678 T20599 T20600 advmod previously,described
R5679 T20600 T20598 amod described,models
R5680 T20601 T20589 punct .,are
R5681 T20603 T20604 det An,mouse
R5682 T20604 T20606 nsubjpass mouse,created
R5683 T20605 T20604 compound ENU,mouse
R5684 T20607 T20604 acl resembling,mouse
R5685 T20608 T20609 amod human,MSUD
R5686 T20609 T20607 dobj MSUD,resembling
R5687 T20610 T20606 auxpass was,created
R5688 T20611 T20606 advmod previously,created
R5689 T20612 T20606 prep by,created
R5690 T20613 T20612 pcomp disrupting,by
R5691 T20614 T20615 det the,gene
R5692 T20615 T20613 dobj gene,disrupting
R5693 T20616 T20615 amod mitochondrial,gene
R5694 T20617 T20615 compound BCAT,gene
R5695 T20618 T20619 punct [,20
R5696 T20619 T20606 parataxis 20,created
R5697 T20620 T20619 punct ],20
R5698 T20621 T20606 punct .,created
R5699 T20623 T20624 det The,activity
R5700 T20624 T20626 nsubj activity,was
R5701 T20625 T20624 compound BCKDH,activity
R5702 T20627 T20624 prep in,activity
R5703 T20628 T20629 det the,liver
R5704 T20629 T20627 pobj liver,in
R5705 T20630 T20629 cc and,liver
R5706 T20631 T20629 conj muscle,liver
R5707 T20632 T20629 prep of,liver
R5708 T20633 T20634 det this,mouse
R5709 T20634 T20632 pobj mouse,of
R5710 T20635 T20626 acomp normal,was
R5711 T20636 T20637 advmod even,were
R5712 T20637 T20626 advcl were,was
R5713 T20638 T20637 mark though,were
R5714 T20639 T20640 det the,levels
R5715 T20640 T20637 nsubj levels,were
R5716 T20641 T20640 compound blood,levels
R5717 T20642 T20640 compound BCAA,levels
R5718 T20643 T20644 advmod markedly,elevated
R5719 T20644 T20637 acomp elevated,were
R5720 T20645 T20626 punct .,was
R5721 T20647 T20648 mark While,is
R5722 T20648 T20650 advcl is,is
R5723 T20649 T20648 nsubj this,is
R5724 T20651 T20652 det an,model
R5725 T20652 T20648 attr model,is
R5726 T20653 T20652 amod interesting,model
R5727 T20654 T20650 punct ", ",is
R5728 T20655 T20650 nsubj it,is
R5729 T20656 T20650 neg not,is
R5730 T20657 T20658 det a,model
R5731 T20658 T20650 attr model,is
R5732 T20659 T20658 amod true,model
R5733 T20660 T20658 prep of,model
R5734 T20661 T20660 pobj MSUD,of
R5735 T20662 T20663 mark because,is
R5736 T20663 T20650 advcl is,is
R5737 T20664 T20665 det the,mutation
R5738 T20665 T20663 nsubj mutation,is
R5739 T20666 T20663 neg not,is
R5740 T20667 T20663 prep in,is
R5741 T20668 T20667 pobj BCKDH,in
R5742 T20669 T20663 cc and,is
R5743 T20670 T20671 det the,levels
R5744 T20671 T20672 nsubj levels,are
R5745 T20672 T20663 conj are,is
R5746 T20673 T20671 prep of,levels
R5747 T20674 T20673 pobj BCKDH,of
R5748 T20675 T20672 acomp normal,are
R5749 T20676 T20650 punct .,is
R5750 T20678 T20679 advmod Furthermore,described
R5751 T20680 T20679 punct ", ",described
R5752 T20681 T20682 det no,case
R5753 T20682 T20679 nsubjpass case,described
R5754 T20683 T20682 prep of,case
R5755 T20790 T20789 compound MSUD,models
R5756 T20791 T20789 compound mouse,models
R5757 T20792 T20793 aux to,test
R5758 T20793 T20785 xcomp test,is
R5759 T20794 T20795 amod novel,treatments
R5760 T20795 T20793 dobj treatments,test
R5761 T20796 T20797 amod such,as
R5762 T20797 T20795 prep as,treatments
R5763 T20798 T20799 npadvmod gene,based
R5764 T20799 T20807 amod based,therapies
R5765 T20800 T20801 punct [,38
R5766 T20801 T20798 parataxis 38,gene
R5767 T20802 T20803 punct -,41
R5768 T20803 T20801 prep 41,38
R5769 T20804 T20801 punct ],38
R5770 T20805 T20798 cc and,gene
R5771 T20806 T20798 conj cell,gene
R5772 T20684 T20683 pobj MSUD,of
R5773 T20807 T20797 pobj therapies,as
R5774 T20685 T20679 aux has,described
R5775 T20686 T20679 auxpass been,described
R5776 T20808 T20809 punct (,hepatocytes
R5777 T20687 T20679 advcl attributing,described
R5778 T20688 T20689 det this,disease
R5779 T20809 T20807 parataxis hepatocytes,therapies
R5780 T20689 T20687 dobj disease,attributing
R5781 T20690 T20687 prep to,attributing
R5782 T20691 T20692 compound BCAT,deficiency
R5783 T20810 T20809 advmod e.g.,hepatocytes
R5784 T20692 T20690 pobj deficiency,to
R5785 T20693 T20679 punct .,described
R5786 T20811 T20809 punct ", ",hepatocytes
R5787 T20695 T20696 prep In,described
R5788 T20812 T20813 punct [,43
R5789 T20697 T20695 pobj addition,In
R5790 T20698 T20697 prep to,addition
R5791 T20813 T20809 parataxis 43,hepatocytes
R5792 T20699 T20700 det this,model
R5793 T20700 T20698 pobj model,to
R5794 T20701 T20700 compound mouse,model
R5795 T20702 T20696 punct ", ",described
R5796 T20814 T20813 nummod 42,43
R5797 T20703 T20704 det a,model
R5798 T20704 T20696 nsubjpass model,described
R5799 T20705 T20704 compound cow,model
R5800 T20706 T20704 prep of,model
R5801 T20707 T20706 pobj MSUD,of
R5802 T20708 T20696 aux has,described
R5803 T20815 T20813 punct ",",43
R5804 T20709 T20696 advmod also,described
R5805 T20710 T20696 auxpass been,described
R5806 T20711 T20696 advmod previously,described
R5807 T20816 T20813 punct ],43
R5808 T20712 T20713 punct [,17
R5809 T20713 T20696 parataxis 17,described
R5810 T20714 T20715 punct -,19
R5811 T20817 T20809 cc or,hepatocytes
R5812 T20715 T20713 prep 19,17
R5813 T20716 T20713 punct ],17
R5814 T20717 T20696 punct .,described
R5815 T20818 T20819 amod embryonic,cells
R5816 T20719 T20720 advmod However,is
R5817 T20819 T20809 conj cells,hepatocytes
R5818 T20721 T20720 punct ", ",is
R5819 T20722 T20720 prep due,is
R5820 T20723 T20722 pcomp to,due
R5821 T20724 T20725 amod practical,constraints
R5822 T20820 T20819 compound stem,cells
R5823 T20725 T20722 pobj constraints,due
R5824 T20726 T20725 acl imposed,constraints
R5825 T20821 T20822 punct [,44
R5826 T20727 T20726 agent by,imposed
R5827 T20728 T20729 amod such,model
R5828 T20729 T20727 pobj model,by
R5829 T20822 T20819 parataxis 44,cells
R5830 T20730 T20729 det a,model
R5831 T20731 T20729 amod large,model
R5832 T20732 T20729 compound animal,model
R5833 T20823 T20822 punct ],44
R5834 T20733 T20722 cc and,due
R5835 T20734 T20722 conj due,due
R5836 T20735 T20734 pcomp to,due
R5837 T20824 T20809 punct ),hepatocytes
R5838 T20736 T20737 det the,observation
R5839 T20737 T20734 pobj observation,due
R5840 T20738 T20737 prep of,observation
R5841 T20825 T20785 punct .,is
R5842 T20739 T20738 pobj differences,of
R5843 T20740 T20739 prep between,differences
R5844 T20827 T20828 advcl Relevant,highlight
R5845 T20741 T20742 det the,model
R5846 T20742 T20740 pobj model,between
R5847 T20829 T20827 prep to,Relevant
R5848 T20743 T20742 compound MSUD,model
R5849 T20744 T20742 compound cow,model
R5850 T20745 T20742 cc and,model
R5851 T20746 T20747 compound MSUD,humans
R5852 T20747 T20742 conj humans,model
R5853 T20748 T20749 punct [,1
R5854 T20749 T20734 parataxis 1,due
R5855 T20830 T20831 compound gene,therapy
R5856 T20750 T20749 punct ],1
R5857 T20751 T20720 punct ", ",is
R5858 T20752 T20753 det this,model
R5859 T20831 T20829 pobj therapy,to
R5860 T20753 T20720 nsubj model,is
R5861 T20754 T20720 advmod also,is
R5862 T20755 T20720 prep of,is
R5863 T20832 T20828 punct ", ",highlight
R5864 T20756 T20757 amod limited,utility
R5865 T20833 T20834 amod recent,problems
R5866 T20757 T20755 pobj utility,of
R5867 T20758 T20720 punct .,is
R5868 T20760 T20761 prep In,are
R5869 T20834 T20828 nsubj problems,highlight
R5870 T20762 T20760 pobj contrast,In
R5871 T20763 T20761 punct ", ",are
R5872 T20835 T20834 prep with,problems
R5873 T20764 T20765 det the,models
R5874 T20765 T20761 nsubj models,are
R5875 T20766 T20765 amod murine,models
R5876 T20836 T20837 compound gene,therapy
R5877 T20767 T20765 acl described,models
R5878 T20768 T20767 prep in,described
R5879 T20769 T20770 det this,report
R5880 T20837 T20835 pobj therapy,with
R5881 T20770 T20768 pobj report,in
R5882 T20771 T20772 advmod more,valid
R5883 T20772 T20761 acomp valid,are
R5884 T20838 T20834 prep in,problems
R5885 T20773 T20772 cc and,valid
R5886 T20774 T20772 conj appropriate,valid
R5887 T20775 T20774 prep for,appropriate
R5888 T20839 T20838 pobj humans,in
R5889 T20776 T20777 amod modeling,MSUD
R5890 T20777 T20775 pobj MSUD,for
R5891 T20778 T20777 amod human,MSUD
R5892 T20840 T20841 det the,importance
R5893 T20779 T20761 punct .,are
R5894 T20841 T20828 dobj importance,highlight
R5895 T20781 T20782 det The,opportunity
R5896 T20782 T20785 nsubj opportunity,is
R5897 T20783 T20784 advmod most,significant
R5898 T20784 T20782 amod significant,opportunity
R5899 T20842 T20841 cc and,importance
R5900 T20786 T20782 acl presented,opportunity
R5901 T20787 T20786 agent by,presented
R5902 T20788 T20789 det the,models
R5903 T20789 T20787 pobj models,by
R5904 T20843 T20844 amod critical,need
R5905 T20844 T20841 conj need,importance
R5906 T20845 T20841 prep of,importance
R5907 T20846 T20847 compound animal,models
R5908 T20847 T20845 pobj models,of
R5909 T20848 T20847 prep of,models
R5910 T20896 T20892 auxpass is,expected
R5911 T20897 T20898 aux to,advance
R5912 T20849 T20850 amod genetic,diseases
R5913 T20898 T20892 xcomp advance,expected
R5914 T20899 T20900 amod such,approaches
R5915 T20900 T20898 dobj approaches,advance
R5916 T20850 T20848 pobj diseases,of
R5917 T20901 T20900 amod therapeutic,approaches
R5918 T20902 T20892 cc and,expected
R5919 T20903 T20904 aux could,pave
R5920 T20904 T20892 conj pave,expected
R5921 T20851 T20841 prep for,importance
R5922 T20905 T20906 det the,way
R5923 T20906 T20904 dobj way,pave
R5924 T20852 T20853 amod preclinical,studies
R5925 T20907 T20904 prep for,pave
R5926 T20908 T20907 pobj studies,for
R5927 T20853 T20851 pobj studies,for
R5928 T20909 T20908 prep in,studies
R5929 T20910 T20909 pobj humans,in
R5930 T20911 T20892 punct .,expected
R5931 T20854 T20828 punct .,highlight
R5932 T20913 T20914 prep In,be
R5933 T20856 T20857 det The,testing
R5934 T20857 T20858 nsubj testing,provides
R5935 T20915 T20913 pobj addition,In
R5936 T20916 T20915 prep to,addition
R5937 T20859 T20857 prep of,testing
R5938 T20917 T20916 pcomp testing,to
R5939 T20918 T20919 amod various,treatments
R5940 T20919 T20917 dobj treatments,testing
R5941 T20920 T20914 punct ", ",be
R5942 T20860 T20861 npadvmod gene,based
R5943 T20921 T20922 det these,models
R5944 T20922 T20914 nsubj models,be
R5945 T20923 T20914 aux should,be
R5946 T20861 T20864 amod based,therapies
R5947 T20924 T20914 advmod also,be
R5948 T20925 T20926 advmod very,useful
R5949 T20862 T20860 cc and,gene
R5950 T20926 T20914 acomp useful,be
R5951 T20927 T20926 prep for,useful
R5952 T20863 T20860 conj cell,gene
R5953 T20928 T20927 pcomp investigating,for
R5954 T20929 T20930 det the,consequences
R5955 T20930 T20928 dobj consequences,investigating
R5956 T20931 T20930 amod underlying,consequences
R5957 T20932 T20930 amod pathophysiologic,consequences
R5958 T20933 T20930 prep of,consequences
R5959 T20864 T20859 pobj therapies,of
R5960 T20934 T20935 det the,disease
R5961 T20935 T20933 pobj disease,of
R5962 T20936 T20914 punct .,be
R5963 T20865 T20857 prep on,testing
R5964 T20938 T20939 amod Such,studies
R5965 T20939 T20940 nsubj studies,are
R5966 T20866 T20867 amod appropriate,systems
R5967 T20941 T20942 advmod very,difficult
R5968 T20942 T20943 amod difficult,impossible
R5969 T20867 T20865 pobj systems,on
R5970 T20943 T20940 acomp impossible,are
R5971 T20944 T20943 punct /,impossible
R5972 T20945 T20946 aux to,do
R5973 T20868 T20867 compound animal,systems
R5974 T20946 T20943 advcl do,impossible
R5975 T20947 T20946 prep in,do
R5976 T20948 T20949 amod human,patients
R5977 T20869 T20867 compound model,systems
R5978 T20949 T20947 pobj patients,in
R5979 T20950 T20946 prep for,do
R5980 T20951 T20952 amod obvious,reasons
R5981 T20870 T20871 det a,method
R5982 T20952 T20950 pobj reasons,for
R5983 T20953 T20952 amod ethical,reasons
R5984 T20954 T20940 punct ", ",are
R5985 T20871 T20858 dobj method,provides
R5986 T20955 T20940 cc and,are
R5987 T20956 T20957 advmod often,rely
R5988 T20957 T20940 conj rely,are
R5989 T20872 T20871 amod preliminary,method
R5990 T20958 T20957 aux must,rely
R5991 T20959 T20957 prep on,rely
R5992 T20960 T20961 compound autopsy,tissue
R5993 T20873 T20871 prep of,method
R5994 T20961 T20959 pobj tissue,on
R5995 T20962 T20940 punct .,are
R5996 T20874 T20873 pcomp establishing,of
R5997 T20964 T20965 advmod Additionally,be
R5998 T20875 T20876 preconj not,efficacy
R5999 T20966 T20965 punct ", ",be
R6000 T20967 T20968 det the,model
R6001 T20968 T20965 nsubj model,be
R6002 T20969 T20968 amod intermediate,model
R6003 T20876 T20874 dobj efficacy,establishing
R6004 T20970 T20968 compound MSUD,model
R6005 T20971 T20965 aux may,be
R6006 T20877 T20875 advmod only,not
R6007 T20972 T20965 advmod also,be
R6008 T20973 T20965 acomp useful,be
R6009 T20974 T20973 prep for,useful
R6010 T20975 T20974 pobj studies,for
R6011 T20976 T20977 aux to,test
R6012 T20977 T20975 advcl test,studies
R6013 T20878 T20876 det the,efficacy
R6014 T20978 T20979 det the,effect
R6015 T20979 T20977 dobj effect,test
R6016 T20980 T20979 prep of,effect
R6017 T20879 T20876 punct ", ",efficacy
R6018 T20981 T20980 pobj thiamin,of
R6019 T20982 T20965 punct .,be
R6020 T20880 T20876 cc but,efficacy
R6021 T20984 T20985 det A,form
R6022 T20881 T20880 advmod also,but
R6023 T20985 T20989 nsubjpass form,described
R6024 T20882 T20883 amod short,term
R6025 T20986 T20987 npadvmod thiamin,responsive
R6026 T20987 T20985 amod responsive,form
R6027 T20883 T20888 compound term,safety
R6028 T20988 T20987 punct -,responsive
R6029 T20990 T20985 prep of,form
R6030 T20884 T20882 punct -,short
R6031 T20991 T20990 pobj MSUD,of
R6032 T20992 T20989 aux has,described
R6033 T20885 T20882 cc and,short
R6034 T20993 T20989 auxpass been,described
R6035 T20994 T20995 punct [,1
R6036 T20886 T20882 conj long,short
R6037 T20995 T20989 parataxis 1,described
R6038 T20996 T20995 punct ],1
R6039 T20997 T20989 punct .,described
R6040 T20887 T20883 punct -,term
R6041 T20999 T21000 det The,phenotype
R6042 T21000 T21001 nsubj phenotype,is
R6043 T20888 T20876 conj safety,efficacy
R6044 T20889 T20858 punct .,provides
R6045 T20891 T20892 nsubjpass Success,expected
R6046 T20893 T20891 prep with,Success
R6047 T21002 T21000 prep of,phenotype
R6048 T21003 T21004 det these,patients
R6049 T20894 T20895 compound animal,studies
R6050 T21004 T21002 pobj patients,of
R6051 T20895 T20893 pobj studies,with
R6052 T21005 T21001 acomp heterogeneous,is
R6053 T21006 T21001 cc and,is
R6055 T21007 T21008 nsubj treatment,produced
R6056 T21008 T21001 conj produced,is
R6057 T21009 T21007 prep with,treatment
R6058 T21010 T21011 det a,range
R6059 T21011 T21009 pobj range,with
R6060 T21012 T21011 amod wide,range
R6061 T21013 T21011 prep of,range
R6062 T21014 T21015 compound thiamin,doses
R6064 T21015 T21013 pobj doses,of
R6065 T21016 T21008 aux has,produced
R6066 T21017 T21018 amod limited,success
R6067 T21018 T21008 dobj success,produced
R6069 T21019 T21020 punct [,1
R6070 T21020 T21008 parataxis 1,produced
R6072 T21021 T21020 punct ],1
R6073 T21022 T21008 punct .,produced
R6075 T21024 T21025 amod Recent,studies
R6076 T21025 T21028 nsubj studies,suggested
R6077 T21026 T21027 compound cell,culture
R6079 T21027 T21025 compound culture,studies
R6080 T21029 T21025 prep with,studies
R6082 T21030 T21031 compound MSUD,cells
R6083 T21031 T21029 pobj cells,with
R6084 T21032 T21028 aux have,suggested
R6086 T21033 T21034 mark that,is
R6087 T21034 T21028 ccomp is,suggested
R6089 T21035 T21036 det the,phenotype
R6090 T21036 T21034 nsubj phenotype,is
R6091 T21117 T21098 punct ", ",provide
R6092 T21037 T21038 npadvmod thiamin,responsive
R6093 T21038 T21036 amod responsive,phenotype
R6094 T21118 T21119 det the,model
R6095 T21039 T21038 punct -,responsive
R6096 T21040 T21034 acomp dependent,is
R6097 T21041 T21040 prep upon,dependent
R6098 T21119 T21098 nsubj model,provide
R6099 T21042 T21043 det the,presence
R6100 T21043 T21041 pobj presence,upon
R6101 T21044 T21043 prep of,presence
R6102 T21045 T21046 advmod at,one
R6103 T21120 T21119 amod intermediate,model
R6104 T21046 T21048 nummod one,allele
R6105 T21047 T21046 advmod least,one
R6106 T21048 T21044 pobj allele,of
R6107 T21121 T21119 compound MSUD,model
R6108 T21049 T21050 npadvmod E2,expressing
R6109 T21050 T21048 amod expressing,allele
R6110 T21051 T21052 punct [,46
R6111 T21052 T21028 parataxis 46,suggested
R6112 T21053 T21052 nummod 45,46
R6113 T21054 T21052 punct ",",46
R6114 T21055 T21052 punct ],46
R6115 T21122 T21098 aux may,provide
R6116 T21056 T21028 punct .,suggested
R6117 T21058 T21059 prep In,provides
R6118 T21123 T21124 det a,resource
R6119 T21060 T21058 pobj light,In
R6120 T21061 T21060 prep of,light
R6121 T21124 T21098 dobj resource,provide
R6122 T21062 T21063 det these,findings
R6123 T21063 T21061 pobj findings,of
R6124 T21064 T21063 amod newer,findings
R6125 T21125 T21124 amod valuable,resource
R6126 T21065 T21059 punct ", ",provides
R6127 T21066 T21067 poss our,mouse
R6128 T21126 T21124 prep for,resource
R6129 T21067 T21059 nsubj mouse,provides
R6130 T21068 T21067 amod intermediate,mouse
R6131 T21069 T21067 compound MSUD,mouse
R6132 T21127 T21126 pobj studies,for
R6133 T21070 T21071 det a,model
R6134 T21071 T21059 dobj model,provides
R6135 T21072 T21073 aux to,test
R6136 T21073 T21071 advcl test,model
R6137 T21128 T21127 prep of,studies
R6138 T21074 T21075 det the,effect
R6139 T21075 T21073 dobj effect,test
R6140 T21076 T21075 prep of,effect
R6141 T21129 T21130 amod structural,biology
R6142 T21077 T21076 pobj thiamin,of
R6143 T21130 T21128 pobj biology,of
R6144 T21078 T21073 prep with,test
R6145 T21079 T21078 pobj respect,with
R6146 T21080 T21079 prep to,respect
R6147 T21081 T21082 compound BCKDH,activity
R6148 T21131 T21098 punct .,provide
R6149 T21082 T21080 pobj activity,to
R6150 T21083 T21082 cc and,activity
R6151 T21084 T21085 compound blood,levels
R6152 T21133 T21134 advmod Lastly,be
R6153 T21085 T21082 conj levels,activity
R6154 T21086 T21087 compound amino,acid
R6155 T21087 T21085 compound acid,levels
R6156 T21088 T21073 prep at,test
R6157 T21135 T21134 punct ", ",be
R6158 T21089 T21090 det the,level
R6159 T21090 T21088 pobj level,at
R6160 T21136 T21137 det the,information
R6161 T21091 T21090 prep of,level
R6162 T21092 T21093 det the,animal
R6163 T21093 T21091 pobj animal,of
R6164 T21094 T21093 amod whole,animal
R6165 T21095 T21059 punct .,provides
R6166 T21137 T21134 nsubj information,be
R6167 T21097 T21098 prep Because,provide
R6168 T21138 T21137 cc and,information
R6169 T21099 T21097 pcomp of,Because
R6170 T21100 T21101 amod recent,interest
R6171 T21101 T21097 pobj interest,Because
R6172 T21139 T21137 conj knowledge,information
R6173 T21102 T21101 prep in,interest
R6174 T21103 T21104 amod structural,analysis
R6175 T21104 T21102 pobj analysis,in
R6176 T21140 T21137 acl gained,information
R6177 T21105 T21104 prep of,analysis
R6178 T21106 T21107 compound multienzyme,complexes
R6179 T21107 T21105 pobj complexes,of
R6180 T21141 T21140 prep from,gained
R6181 T21142 T21141 pobj studies,from
R6182 T21143 T21142 prep with,studies
R6183 T21144 T21145 det the,models
R6184 T21145 T21143 pobj models,with
R6185 T21146 T21145 compound MSUD,models
R6186 T21147 T21145 acl described,models
R6187 T21148 T21147 advmod here,described
R6188 T21149 T21134 aux will,be
R6189 T21150 T21134 advmod also,be
R6190 T21151 T21134 acomp applicable,be
R6191 T21152 T21151 cc and,applicable
R6192 T21153 T21151 conj transferable,applicable
R6193 T21154 T21153 prep to,transferable
R6194 T21155 T21156 amod other,disorders
R6195 T21156 T21154 pobj disorders,to
R6196 T21157 T21156 amod mitochondrial,disorders
R6197 T21158 T21134 prep due,be
R6198 T21159 T21158 pcomp to,due
R6199 T21160 T21158 pobj defects,due
R6200 T21161 T21160 prep in,defects
R6201 T21162 T21163 amod multisubunit,enzymes
R6202 T21163 T21161 pobj enzymes,in
R6203 T21164 T21134 punct .,be
R6054 T21108 T21109 punct [,46
R6063 T21109 T21097 parataxis 46,Because
R6068 T21110 T21109 nummod 35,46
R6071 T21111 T21109 punct ",",46
R6074 T21112 T21109 nummod 36,46
R6078 T21113 T21109 punct ",",46
R6081 T21114 T21109 nummod 45,46
R6085 T21115 T21109 punct ",",46
R6088 T21116 T21109 punct ],46

craft-ca-core-ex-dev

Below, discontinuous spans are shown in the chain model. You can change it to the bag model.

Id Subject Object Predicate Lexical cue
T19825 7128-7135 CHEBI_GO_SO_EXT:enzyme denotes enzymes
T19824 7073-7086 GO:0005739 denotes mitochondrial
T19823 7051-7063 GO_EXT:biological_movement_or_translocation_process denotes transferable
T19822 6920-6927 CHEBI_GO_EXT:biological_process_or_quality_or_role denotes biology
T19821 6809-6818 GO:0032991 denotes complexes
T19820 6802-6808 CHEBI_GO_SO_EXT:enzyme denotes enzyme
T19819 6736-6742 NCBITaxon:33208 denotes animal
T19818 6692-6702 CHEBI_SO_EXT:amino_acid denotes amino acid
T19817 6686-6691 UBERON:0000178 denotes blood
T19816 6643-6650 CHEBI:26948 denotes thiamin
T19815 6598-6603 NCBITaxon:10088 denotes mouse
T19814 6526-6532 SO_EXT:0001023 denotes allele
T19813 6515-6525 GO:0010467 denotes expressing
T19812 6512-6514 PR_EXT:000006300 denotes E2
T19811 6436-6443 CHEBI:26948 denotes thiamin
T19810 6406-6411 CL_GO_EXT:cell denotes cells
T19809 6375-6379 CL_GO_EXT:cell denotes cell
T19808 6320-6327 CHEBI:26948 denotes thiamin
T19807 6180-6187 CHEBI:26948 denotes thiamin
T19806 6169-6176 CHEBI:26948 denotes thiamin
T19805 6066-6072 UBERON:0000479 denotes tissue
T19804 5991-5996 NCBITaxon:9606 denotes human
T19803 5769-5775 NCBITaxon:9606 denotes humans
T19802 5665-5671 NCBITaxon:33208 denotes animal
T19801 5524-5530 NCBITaxon:33208 denotes animal
T19800 5488-5492 CL_GO_EXT:cell denotes cell
T19799 5479-5483 SO_EXT:0000704 denotes gene
T19798 5422-5429 SO_EXT:0000704 denotes genetic
T19797 5405-5411 NCBITaxon:33208 denotes animal
T19796 5352-5358 NCBITaxon:9606 denotes humans
T19795 5336-5340 SO_EXT:0000704 denotes gene
T19794 5301-5305 SO_EXT:0000704 denotes gene
T19793 5276-5281 CL_GO_EXT:cell denotes cells
T19792 5261-5281 CL:0002322 denotes embryonic stem cells
T19791 5261-5270 UBERON:0000922 denotes embryonic
T19790 5238-5249 CL:0000182 denotes hepatocytes
T19789 5210-5214 CL_GO_EXT:cell denotes cell
T19788 5193-5197 SO_EXT:0000704 denotes gene
T19787 5144-5149 NCBITaxon:10088 denotes mouse
T19786 5077-5082 NCBITaxon:9606 denotes human
T19785 4994-5000 NCBITaxon:39107 denotes murine
T19784 4926-4932 NCBITaxon:9606 denotes humans
T19783 4907-4910 NCBITaxon:9913 denotes cow
T19782 4835-4841 NCBITaxon:33208 denotes animal
T19781 4711-4714 NCBITaxon:9913 denotes cow
T19780 4696-4701 NCBITaxon:10088 denotes mouse
T19779 4659-4663 GO_PR_EXT:branched_chain_amino_acid_aminotransferase denotes BCAT
T19778 4522-4530 SO_EXT:sequence_alteration_entity_or_process denotes mutation
T19777 4407-4411 CHEBI:22918 denotes BCAA
T19776 4401-4406 UBERON:0000178 denotes blood
T19775 4368-4373 NCBITaxon:10088 denotes mouse
T19774 4353-4359 UBERON_EXT:muscle_structure_or_tissue denotes muscle
T19773 4343-4348 UBERON:0002107 denotes liver
T19772 4306-4310 SO_EXT:0000704 denotes gene
T19771 4301-4305 GO_PR_EXT:branched_chain_amino_acid_aminotransferase denotes BCAT
T19770 4287-4305 PR_EXT:000004683 denotes mitochondrial BCAT
T19769 4287-4300 GO:0005739 denotes mitochondrial
T19768 4235-4240 NCBITaxon:9606 denotes human
T19767 4218-4223 NCBITaxon:10088 denotes mouse
T19766 4214-4217 CHEBI:23995 denotes ENU
T19765 4096-4105 GO:0065007 denotes regulated
T19764 3986-3996 GO:0010467 denotes expression
T19763 3976-3985 GO:0065007 denotes regulated
T19762 3942-3952 SO_EXT:0000902 denotes transgenes
T19761 3918-3934 SO_EXT:0000366 denotes integration site
T19760 3867-3878 CHEBI:50845 denotes doxycycline
T19759 3777-3787 SO_EXT:transgenic_entity denotes transgenic
T19758 3744-3755 CHEBI:50845 denotes doxycycline
T19757 3721-3733 CHEBI:27902 denotes tetracycline
T19756 3698-3706 SO_EXT:0005853 denotes cassette
T19755 3687-3697 GO:0010467 denotes expression
T19754 3684-3686 PR_EXT:000006300 denotes E2
T19753 3678-3683 NCBITaxon:9606 denotes human
T19752 3614-3623 SO_EXT:0000902 denotes transgene
T19751 3605-3613 SO_EXT:sequence_rescue_process denotes rescuing
T19750 3463-3467 NCBITaxon:10088 denotes mice
T19749 3323-3327 SO_EXT:0000704 denotes gene
T19748 3313-3322 GO:0065007 denotes regulated
T19747 3300-3312 CHEBI:27902 denotes tetracycline
T19746 3211-3221 SO_EXT:transgenic_entity denotes transgenic
T19745 3160-3166 NCBITaxon:33208 denotes animal
T19744 3130-3134 NCBITaxon:10088 denotes mice
T19743 3107-3118 SO_EXT:0000704 denotes genetically
T19742 3085-3090 NCBITaxon:9606 denotes human
T19741 3073-3077 NCBITaxon:10088 denotes mice
T19740 3066-3072 SO_EXT:sequence_rescue_process denotes rescue
T19739 2959-2963 CHEBI:22918 denotes BCAA
T19738 2907-2912 NCBITaxon:9606 denotes human
T19737 2817-2821 NCBITaxon:10088 denotes mice
T19736 2810-2816 SO_EXT:sequence_rescue_process denotes rescue
T19735 2795-2800 UBERON:0000178 denotes blood
T19734 2780-2784 CHEBI:22918 denotes BCAA
T19733 2714-2719 UBERON:0002107 denotes liver
T19732 2659-2664 NCBITaxon:10088 denotes mouse
T19731 2608-2617 UBERON:0000113 denotes adulthood
T19730 2591-2595 NCBITaxon:10088 denotes mice
T19729 2584-2590 SO_EXT:sequence_rescue_process denotes rescue
T19728 2561-2570 GO_EXT:fatality_or_lethality denotes lethality
T19727 2552-2560 PATO_UBERON_EXT:neonate_or_newborn denotes neonatal
T19726 2541-2547 SO_EXT:sequence_rescue_process denotes rescue
T19725 2525-2529 NCBITaxon:10088 denotes mice
T19724 2501-2506 UBERON:0002107 denotes liver
T19723 2484-2493 SO_EXT:0000902 denotes transgene
T19722 2481-2483 PR_EXT:000006300 denotes E2
T19721 2475-2480 NCBITaxon:9606 denotes human
T19720 2459-2469 GO:0010467 denotes expression
T19719 2429-2444 UBERON:0007221 denotes neonatal period
T19718 2408-2411 GO:0016265 denotes die
T19717 2405-2407 PR_EXT:000006300 denotes E2
T19716 2387-2396 SO_EXT:0000902 denotes transgene
T19715 2366-2368 PR_EXT:000006300 denotes E2
T19714 2340-2344 NCBITaxon:10088 denotes mice
T19713 2328-2330 PR_EXT:000006300 denotes E2
T19712 2319-2324 UBERON:0002107 denotes liver
T19711 2309-2311 PR_EXT:000006300 denotes E2
T19710 2303-2308 NCBITaxon:9606 denotes human
T19709 2295-2302 GO:0010467 denotes express
T19708 2272-2282 SO_EXT:transgenic_entity denotes transgenic
T19707 2194-2205 CHEBI_EXT:biochemical denotes biochemical
T19706 2156-2161 NCBITaxon:9606 denotes human
T19705 2062-2067 NCBITaxon:10088 denotes mouse
T19704 2053-2061 SO_EXT:sequence_alteration_entity_or_process denotes mutation
T19703 2048-2052 SO_EXT:sequence_nullness denotes null
T19702 1970-1979 CHEBI_GO_SO_EXT:enzyme denotes enzymatic
T19701 1922-1926 CHEBI:22918 denotes BCAA
T19700 1818-1822 NCBITaxon_UBERON_EXT:calf denotes calf
T19699 1751-1756 UBERON:0000955 denotes brain
T19698 1710-1714 CHEBI:22918 denotes BCAA
T19697 1663-1672 GO_EXT:fatality_or_lethality denotes lethality
T19696 1654-1662 PATO_UBERON_EXT:neonate_or_newborn denotes neonatal
T19695 1642-1647 GO:0007567 denotes birth
T19694 1618-1622 GO:0016265 denotes died
T19693 1613-1617 NCBITaxon_UBERON_EXT:pup denotes pups
T19692 1483-1494 CHEBI_SO_EXT:amino_acid denotes amino acids
T19691 1444-1451 CHEBI_SO_EXT:alanine denotes alanine
T19690 1433-1442 CHEBI_SO_EXT:glutamine denotes glutamine
T19689 1422-1431 CHEBI_EXT:glutamate denotes glutamate
T19688 1368-1378 UBERON:0001016 denotes neurologic
T19687 1332-1343 UBERON:0001004 denotes respiratory
T19686 1312-1317 GO_EXT:biological_movement_or_translocation_process denotes motor
T19685 1245-1255 UBERON:0001016 denotes neurologic
T19684 1191-1198 PATO_UBERON_EXT:neonate_or_newborn denotes newborn
T19683 1159-1163 NCBITaxon_UBERON_EXT:pup denotes pups
T19682 1153-1158 NCBITaxon:10088 denotes mouse
T19681 1130-1138 GO_PATO_EXT:biological_behavior denotes behavior
T19680 1109-1113 NCBITaxon_UBERON_EXT:pup denotes pups
T19679 1083-1094 CL:0000057 denotes fibroblasts
T19678 1073-1078 UBERON:0002107 denotes liver
T19677 1051-1058 CHEBI_PR_EXT:protein denotes protein
T19676 1048-1050 PR_EXT:000006300 denotes E2
T19675 1027-1031 CHEBI:22918 denotes BCAA
T19674 1015-1026 GO:0008152 denotes metabolized
T19673 955-959 NCBITaxon_UBERON_EXT:pup denotes pups
T19672 949-954 NCBITaxon:10088 denotes mouse
T19671 919-925 UBERON:0002107 denotes livers
T19670 862-866 CHEBI:22918 denotes BCAA
T19669 848-866 GO:0009081 denotes metabolism of BCAA
T19668 799-808 CHEBI_SO_EXT:glutamine denotes glutamine
T19667 784-793 CHEBI_EXT:glutamate denotes glutamate
T19666 775-782 CHEBI_SO_EXT:alanine denotes alanine
T19665 763-774 CHEBI_SO_EXT:amino_acid denotes amino acids
T19664 705-709 CHEBI:22918 denotes BCAA
T19663 689-694 UBERON:0000178 denotes blood
T19662 679-687 GO:0001967 denotes suckling
T19661 659-664 GO:0007567 denotes birth
T19660 636-641 GO:0007567 denotes birth
T19659 582-586 GO:0007567 denotes born
T19658 572-576 NCBITaxon:10088 denotes mice
T19657 537-543 NCBITaxon:9606 denotes humans
T19656 504-511 NCBITaxon:33208 denotes animals
T19655 451-470 PR_EXT:000006300 denotes E2 subunit of BCKDH
T19654 430-434 SO_EXT:0000704 denotes gene
T19653 266-271 NCBITaxon:10088 denotes mouse
T19652 243-254 SO_EXT:0000704 denotes genetically
T19651 107-113 NCBITaxon:33208 denotes animal

craft-ca-core-dev

Below, discontinuous spans are shown in the chain model. You can change it to the bag model.

Id Subject Object Predicate Lexical cue
T19647 6686-6691 UBERON:0000178 denotes blood
T19646 6643-6650 CHEBI:26948 denotes thiamin
T19645 6598-6603 NCBITaxon:10088 denotes mouse
T19644 6526-6532 SO:0001023 denotes allele
T19643 6515-6525 GO:0010467 denotes expressing
T19642 6512-6514 PR:000006300 denotes E2
T19641 6436-6443 CHEBI:26948 denotes thiamin
T19640 6320-6327 CHEBI:26948 denotes thiamin
T19639 6180-6187 CHEBI:26948 denotes thiamin
T19638 6169-6176 CHEBI:26948 denotes thiamin
T19637 6066-6072 UBERON:0000479 denotes tissue
T19636 5991-5996 NCBITaxon:9606 denotes human
T19635 5769-5775 NCBITaxon:9606 denotes humans
T19634 5665-5671 NCBITaxon:33208 denotes animal
T19633 5524-5530 NCBITaxon:33208 denotes animal
T19632 5479-5483 SO:0000704 denotes gene
T19631 5422-5429 SO:0000704 denotes genetic
T19630 5405-5411 NCBITaxon:33208 denotes animal
T19629 5352-5358 NCBITaxon:9606 denotes humans
T19628 5336-5340 SO:0000704 denotes gene
T19627 5301-5305 SO:0000704 denotes gene
T19626 5261-5281 CL:0002322 denotes embryonic stem cells
T19625 5261-5270 UBERON:0000922 denotes embryonic
T19624 5238-5249 CL:0000182 denotes hepatocytes
T19623 5193-5197 SO:0000704 denotes gene
T19622 5144-5149 NCBITaxon:10088 denotes mouse
T19621 5077-5082 NCBITaxon:9606 denotes human
T19620 4994-5000 NCBITaxon:39107 denotes murine
T19619 4926-4932 NCBITaxon:9606 denotes humans
T19618 4907-4910 NCBITaxon:9913 denotes cow
T19617 4835-4841 NCBITaxon:33208 denotes animal
T19616 4711-4714 NCBITaxon:9913 denotes cow
T19615 4696-4701 NCBITaxon:10088 denotes mouse
T19614 4407-4411 CHEBI:22918 denotes BCAA
T19613 4401-4406 UBERON:0000178 denotes blood
T19612 4368-4373 NCBITaxon:10088 denotes mouse
T19611 4343-4348 UBERON:0002107 denotes liver
T19610 4306-4310 SO:0000704 denotes gene
T19609 4287-4305 PR:000004683 denotes mitochondrial BCAT
T19608 4287-4300 GO:0005739 denotes mitochondrial
T19607 4235-4240 NCBITaxon:9606 denotes human
T19606 4218-4223 NCBITaxon:10088 denotes mouse
T19605 4214-4217 CHEBI:23995 denotes ENU
T19604 4096-4105 GO:0065007 denotes regulated
T19603 3986-3996 GO:0010467 denotes expression
T19602 3976-3985 GO:0065007 denotes regulated
T19601 3942-3952 SO:0000902 denotes transgenes
T19600 3918-3934 SO:0000366 denotes integration site
T19599 3867-3878 CHEBI:50845 denotes doxycycline
T19598 3744-3755 CHEBI:50845 denotes doxycycline
T19597 3721-3733 CHEBI:27902 denotes tetracycline
T19596 3698-3706 SO:0005853 denotes cassette
T19595 3687-3697 GO:0010467 denotes expression
T19594 3684-3686 PR:000006300 denotes E2
T19593 3678-3683 NCBITaxon:9606 denotes human
T19592 3614-3623 SO:0000902 denotes transgene
T19591 3463-3467 NCBITaxon:10088 denotes mice
T19590 3323-3327 SO:0000704 denotes gene
T19589 3313-3322 GO:0065007 denotes regulated
T19588 3300-3312 CHEBI:27902 denotes tetracycline
T19587 3160-3166 NCBITaxon:33208 denotes animal
T19586 3130-3134 NCBITaxon:10088 denotes mice
T19585 3107-3118 SO:0000704 denotes genetically
T19584 3085-3090 NCBITaxon:9606 denotes human
T19583 3073-3077 NCBITaxon:10088 denotes mice
T19582 2959-2963 CHEBI:22918 denotes BCAA
T19581 2907-2912 NCBITaxon:9606 denotes human
T19580 2817-2821 NCBITaxon:10088 denotes mice
T19579 2795-2800 UBERON:0000178 denotes blood
T19543 1048-1050 PR:000006300 denotes E2
T19542 1027-1031 CHEBI:22918 denotes BCAA
T19541 1015-1026 GO:0008152 denotes metabolized
T19540 949-954 NCBITaxon:10088 denotes mouse
T19539 919-925 UBERON:0002107 denotes livers
T19538 862-866 CHEBI:22918 denotes BCAA
T19537 848-866 GO:0009081 denotes metabolism of BCAA
T19536 705-709 CHEBI:22918 denotes BCAA
T19535 689-694 UBERON:0000178 denotes blood
T19534 679-687 GO:0001967 denotes suckling
T19533 659-664 GO:0007567 denotes birth
T19532 636-641 GO:0007567 denotes birth
T19531 582-586 GO:0007567 denotes born
T19530 572-576 NCBITaxon:10088 denotes mice
T19529 537-543 NCBITaxon:9606 denotes humans
T19528 504-511 NCBITaxon:33208 denotes animals
T19527 451-470 PR:000006300 denotes E2 subunit of BCKDH
T19526 430-434 SO:0000704 denotes gene
T19525 266-271 NCBITaxon:10088 denotes mouse
T19524 243-254 SO:0000704 denotes genetically
T19523 107-113 NCBITaxon:33208 denotes animal
T19650 7073-7086 GO:0005739 denotes mitochondrial
T19649 6809-6818 GO:0032991 denotes complexes
T19648 6736-6742 NCBITaxon:33208 denotes animal
T19578 2780-2784 CHEBI:22918 denotes BCAA
T19577 2714-2719 UBERON:0002107 denotes liver
T19576 2659-2664 NCBITaxon:10088 denotes mouse
T19575 2608-2617 UBERON:0000113 denotes adulthood
T19574 2591-2595 NCBITaxon:10088 denotes mice
T19573 2525-2529 NCBITaxon:10088 denotes mice
T19572 2501-2506 UBERON:0002107 denotes liver
T19571 2484-2493 SO:0000902 denotes transgene
T19570 2481-2483 PR:000006300 denotes E2
T19569 2475-2480 NCBITaxon:9606 denotes human
T19568 2459-2469 GO:0010467 denotes expression
T19567 2429-2444 UBERON:0007221 denotes neonatal period
T19566 2408-2411 GO:0016265 denotes die
T19565 2405-2407 PR:000006300 denotes E2
T19564 2387-2396 SO:0000902 denotes transgene
T19563 2366-2368 PR:000006300 denotes E2
T19562 2340-2344 NCBITaxon:10088 denotes mice
T19561 2328-2330 PR:000006300 denotes E2
T19560 2319-2324 UBERON:0002107 denotes liver
T19559 2309-2311 PR:000006300 denotes E2
T19558 2303-2308 NCBITaxon:9606 denotes human
T19557 2295-2302 GO:0010467 denotes express
T19556 2156-2161 NCBITaxon:9606 denotes human
T19555 2062-2067 NCBITaxon:10088 denotes mouse
T19554 1922-1926 CHEBI:22918 denotes BCAA
T19553 1751-1756 UBERON:0000955 denotes brain
T19552 1710-1714 CHEBI:22918 denotes BCAA
T19551 1642-1647 GO:0007567 denotes birth
T19550 1618-1622 GO:0016265 denotes died
T19549 1368-1378 UBERON:0001016 denotes neurologic
T19548 1332-1343 UBERON:0001004 denotes respiratory
T19547 1245-1255 UBERON:0001016 denotes neurologic
T19546 1153-1158 NCBITaxon:10088 denotes mouse
T19545 1083-1094 CL:0000057 denotes fibroblasts
T19544 1073-1078 UBERON:0002107 denotes liver